Reference/ChEBI CHEBI 16015
type: UnificationXref
id: CHEBI:16015
db: ChEBI

Reference/PubChem-compound 439161
type: UnificationXref
id: 439161
db: PubChem-compound

Reference/KEGG Compound C00430
type: UnificationXref
id: C00430
db: KEGG Compound

Glycine--tRNA ligase
type: Protein
cellular location: Cytoplasm
entity reference: Glycine--tRNA ligase

L-Arginine
type: SmallMolecule
cellular location: Mitochondrion
entity reference: L-Arginine

Reference/ChEBI CHEBI 30314
type: UnificationXref
id: CHEBI:30314
db: ChEBI

Ammonia
type: SmallMolecule
cellular location: Mitochondrial Outer Membrane
entity reference: Ammonia

Reference/PubChem-compound 439153
type: UnificationXref
id: 439153
db: PubChem-compound

Reference/ChemSpider 57
type: UnificationXref
id: 57
db: ChemSpider

Reference/ChemSpider 50
type: UnificationXref
id: 50
db: ChemSpider

Reference/ChEBI CHEBI 20506
type: UnificationXref
id: CHEBI:20506
db: ChEBI

Reference/PubChem-compound 53481912
type: UnificationXref
id: 53481912
db: PubChem-compound

Reference/ChEBI CHEBI 17115
type: UnificationXref
id: CHEBI:17115
db: ChEBI

Reference/ChEBI CHEBI 16027
type: UnificationXref
id: CHEBI:16027
db: ChEBI

Sarcosine
type: SmallMolecule
cellular location: Cytosol
entity reference: Sarcosine

Reference/PubChem-compound 6137
type: UnificationXref
id: 6137
db: PubChem-compound

Glycine N-methyltransferase
type: ProteinReference
xref: Reference/UniProt Q14749
organism: Homo sapiens

Reference/ChemSpider 559142
type: UnificationXref
id: 559142
db: ChemSpider

Reference/UniProt P09622
type: UnificationXref
id: P09622
db: UniProt

Reference/ChEBI CHEBI 15380
type: UnificationXref
id: CHEBI:15380
db: ChEBI

Reference/ChemSpider 5742
type: UnificationXref
id: 5742
db: ChemSpider

NADH
type: SmallMolecule
cellular location: Cytosol
entity reference: NADH

Succinyl-CoA
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00091
xref: Reference/CAS 604-98-8
xref: Reference/ChemSpider 388307
xref: Reference/PubChem-compound 439161
xref: Reference/ChEBI CHEBI 15380
xref: Reference/BioCyc 3-METHYLBENZYLSUCCINYL-COA
xref: Reference/HMDB HMDB0001022
chemical formula: C25H40N7O19P3S
molecular weight: 867.1312
structure: ChemicalStructure/PW C000808

Reference/KEGG Compound C00213
type: UnificationXref
id: C00213
db: KEGG Compound

Reference/ChEBI CHEBI 17561
type: UnificationXref
id: CHEBI:17561
db: ChEBI

Reference/PubChem-compound 439175
type: UnificationXref
id: 439175
db: PubChem-compound

Reference/BioCyc L-CYSTATHIONINE
type: UnificationXref
id: L-CYSTATHIONINE
db: BioCyc

Amine oxidase [flavin-containing] A
type: ProteinReference
xref: Reference/UniProt P21397
organism: Homo sapiens

Reference/ChemSpider 5736
type: UnificationXref
id: 5736
db: ChemSpider

Phosphohydroxypyruvic acid
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001024
xref: Reference/PubChem-compound 105
xref: Reference/ChemSpider 103
xref: Reference/CAS 3913-50-6
xref: Reference/ChEBI CHEBI 30933
xref: Reference/KEGG Compound C03232
xref: Reference/BioCyc 3-P-HYDROXYPYRUVATE
chemical formula: C3H5O7P
molecular weight: 183.9773
structure: ChemicalStructure/PW C000810

Reference/ChemSpider 5735
type: UnificationXref
id: 5735
db: ChemSpider

Reference/ChEBI CHEBI 16240
type: UnificationXref
id: CHEBI:16240
db: ChEBI

Reference/PathWhiz PW000152
type: UnificationXref
id: PW000152
db: PathWhiz

ChemicalStructure/PW C001099
type: ChemicalStructure
structure format: SMILES

2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: 2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial

Glycerate kinase
type: Protein
cellular location: Cytosol
entity reference: Glycerate kinase

Serine hydroxymethyltransferase, cytosolic
type: Protein
cellular location: Cytosol
entity reference: Serine hydroxymethyltransferase, cytosolic

Reference/KEGG Compound C03508
type: UnificationXref
id: C03508
db: KEGG Compound

ChemicalStructure/PW C000808
type: ChemicalStructure
structure format: SMILES

Compound/PW C000120 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Serine
stoichiometric coefficient: 1.0

Reference/CAS 3913-50-6
type: UnificationXref
id: 3913-50-6
db: CAS

Reference/CAS 979-92-0
type: UnificationXref
id: 979-92-0
db: CAS

Compound/PW C001144 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: NADH
stoichiometric coefficient: 1.0

Dimethylglycine
type: SmallMolecule
cellular location: Cytosol
entity reference: Dimethylglycine

Serine racemase
type: ProteinReference
xref: Reference/UniProt Q9GZT4
organism: Homo sapiens

Compound/PW C000894 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 5-Aminolevulinic acid
stoichiometric coefficient: 1.0

Compound/PW C000035 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Ammonia
stoichiometric coefficient: 1.0

Reference/KEGG Compound C00009
type: UnificationXref
id: C00009
db: KEGG Compound

Reference/KEGG Compound C00008
type: UnificationXref
id: C00008
db: KEGG Compound

Reference/KEGG Compound C00007
type: UnificationXref
id: C00007
db: KEGG Compound

Reference/ChEBI CHEBI 17158
type: UnificationXref
id: CHEBI:17158
db: ChEBI

Reference/KEGG Compound C00001
type: UnificationXref
id: C00001
db: KEGG Compound

Reference/PubChem-compound 439194
type: UnificationXref
id: 439194
db: PubChem-compound

Reference/BioCyc L-ALPHA-ALANINE
type: UnificationXref
id: L-ALPHA-ALANINE
db: BioCyc

ChemicalStructure/PW C000810
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)C(=O)COP(O)(O)=O

Reference/CAS 56-41-7
type: UnificationXref
id: 56-41-7
db: CAS

Reference/KEGG Compound C00004
type: UnificationXref
id: C00004
db: KEGG Compound

Reference/KEGG Compound C00003
type: UnificationXref
id: C00003
db: KEGG Compound

Reference/CAS 135-16-0
type: UnificationXref
id: 135-16-0
db: CAS

Compound/PW C000062 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Dimethylglycine
stoichiometric coefficient: 1.0

Reference/BioCyc 2-OXOBUTANOATE
type: UnificationXref
id: 2-OXOBUTANOATE
db: BioCyc

Reference/KEGG Compound C00002
type: UnificationXref
id: C00002
db: KEGG Compound

Cystathionine gamma-lyase
type: Complex
component stoichiometry: Protein/511 Cytosol/Stoichiometry/4.0
cellular location: Cytosol
component: Cystathionine gamma-lyase

Coenzyme A
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Coenzyme A

Compound/PW C001435 Mitochondrial Outer Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Aminoacetone
stoichiometric coefficient: 1.0

Mitochondrial Inner Membrane
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005743

Reference/KEGG Compound C00019
type: UnificationXref
id: C00019
db: KEGG Compound

Oxygen
type: SmallMolecule
cellular location: Mitochondrial Outer Membrane
entity reference: Oxygen

Reference/CAS 352-97-6
type: UnificationXref
id: 352-97-6
db: CAS

Compound/PW C000170 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyrophosphate
stoichiometric coefficient: 1.0

Compound/PW C001316 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Carbon dioxide
stoichiometric coefficient: 1.0

Compound/PW C000185 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Sarcosine
stoichiometric coefficient: 1.0

Reference/KEGG Compound C00011
type: UnificationXref
id: C00011
db: KEGG Compound

Reference/KEGG Compound C00010
type: UnificationXref
id: C00010
db: KEGG Compound

Reference/KEGG Compound C00258
type: UnificationXref
id: C00258
db: KEGG Compound

Reference/KEGG Compound C00014
type: UnificationXref
id: C00014
db: KEGG Compound

Reference/KEGG Compound C01103
type: UnificationXref
id: C01103
db: KEGG Compound

Reference/KEGG Compound C00013
type: UnificationXref
id: C00013
db: KEGG Compound

Compound/PW C000078 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Glycine
stoichiometric coefficient: 1.0

Reference/PubChem-compound 6112
type: UnificationXref
id: 6112
db: PubChem-compound

Reference/KEGG Compound C00022
type: UnificationXref
id: C00022
db: KEGG Compound

Reference/KEGG Compound C00021
type: UnificationXref
id: C00021
db: KEGG Compound

Reference/CAS 56-65-5
type: UnificationXref
id: 56-65-5
db: CAS

Reference/BioCyc MET
type: UnificationXref
id: MET
db: BioCyc

Reference/CAS 53-84-9
type: UnificationXref
id: 53-84-9
db: CAS

Reference/KEGG Compound C00020
type: UnificationXref
id: C00020
db: KEGG Compound

Reference/KEGG Compound C00027
type: UnificationXref
id: C00027
db: KEGG Compound

L-Glutamic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: L-Glutamic acid

Reference/KEGG Compound C00026
type: UnificationXref
id: C00026
db: KEGG Compound

Reference/KEGG Compound C00025
type: UnificationXref
id: C00025
db: KEGG Compound

Reference/KEGG Compound C00024
type: UnificationXref
id: C00024
db: KEGG Compound

ChemicalStructure/PW C008526
type: ChemicalStructure
structure format: SMILES
structure data: NCSCCC(S)CCCCC(O)=O

Reference/CAS 3432-99-3
type: UnificationXref
id: 3432-99-3
db: CAS

Glycyl-tRNA
type: Rna
cellular location: Cytoplasm
entity reference: Glycyl-tRNA

Reference/CAS 298-12-4
type: UnificationXref
id: 298-12-4
db: CAS

Reference/CAS 1118-68-9
type: UnificationXref
id: 1118-68-9
db: CAS

Carbon dioxide
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: Carbon dioxide

Reference/UniProt Q9UL12
type: UnificationXref
id: Q9UL12
db: UniProt

Serine--pyruvate aminotransferase
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Serine--pyruvate aminotransferase

D-3-phosphoglycerate dehydrogenase
type: Protein
cellular location: Cytosol
entity reference: D-3-phosphoglycerate dehydrogenase

Reference/CAS 52-90-4
type: UnificationXref
id: 52-90-4
db: CAS

Reference/PubChem-compound 760
type: UnificationXref
id: 760
db: PubChem-compound

Reference/KEGG Compound C00037
type: UnificationXref
id: C00037
db: KEGG Compound

Reference/ChemSpider 653
type: UnificationXref
id: 653
db: ChemSpider

Reference/HMDB HMDB0003406
type: UnificationXref
id: HMDB0003406
db: HMDB

Reference/KEGG Compound C00041
type: UnificationXref
id: C00041
db: KEGG Compound

Reference/HMDB HMDB0000139
type: UnificationXref
id: HMDB0000139
db: HMDB

Compound/PW C000905 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyruvaldehyde
stoichiometric coefficient: 1.0

ChemicalStructure/PW C000170
type: ChemicalStructure
structure format: SMILES
structure data: OP(O)(=O)OP(O)(O)=O

Reference/ChemSpider 408
type: UnificationXref
id: 408
db: ChemSpider

Reference/ChEBI CHEBI 18047
type: UnificationXref
id: CHEBI:18047
db: ChEBI

Reference/KEGG Compound C00048
type: UnificationXref
id: C00048
db: KEGG Compound

Reference/CAS 107-97-1
type: UnificationXref
id: 107-97-1
db: CAS

Orotidylic acid
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Orotidylic acid

ChemicalStructure/PW C001034
type: ChemicalStructure
structure format: SMILES

L-serine dehydratase/L-threonine deaminase
type: Protein
cellular location: Cytosol
entity reference: L-serine dehydratase/L-threonine deaminase

ChemicalStructure/PW C000186
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](COP(O)(O)=O)C(O)=O

ChemicalStructure/PW C000185
type: ChemicalStructure
structure format: SMILES
structure data: CNCC(O)=O

Phosphoserine aminotransferase
type: ProteinReference
xref: Reference/UniProt Q9Y617
organism: Homo sapiens

Reference/CAS 604-98-8
type: UnificationXref
id: 604-98-8
db: CAS

Reference/HMDB HMDB0000148
type: UnificationXref
id: HMDB0000148
db: HMDB

Compound/PW C000134 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Oxoglutaric acid
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 29162
type: UnificationXref
id: CHEBI:29162
db: ChEBI

L-Seryl-tRNA(Ser)
type: RnaReference
xref: Reference/ChEBI CHEBI 29162
organism: Homo sapiens

Glycyl-tRNA
type: RnaReference
xref: Reference/ChEBI CHEBI 29156
organism: Homo sapiens

L-Seryl-tRNA
type: RnaReference
xref: Reference/ChEBI CHEBI 29162
organism: Homo sapiens

Glycyl-tRNA(Gly)
type: RnaReference
xref: Reference/ChEBI CHEBI 29156
organism: Homo sapiens

4.4.1.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000003 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000035 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000067 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000448 Cytosol/Stoichiometry/1.0
right: L-Cysteine
right: Ammonia
right: 2-Ketobutyric acid
spontaneous: false
EC number: 4.4.1.1
left: L-Cystathionine
left: Water
name: L-Cystathionine + Water → 2-Ketobutyric acid + Ammonia + L-Cysteine
conversion direction: LEFT_TO_RIGHT

Reference/PubChem-compound 784
type: UnificationXref
id: 784
db: PubChem-compound

Reaction815
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000120 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000067 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000590 Cytosol/Stoichiometry/1.0
right: Water
right: L-Cystathionine
spontaneous: false
left: L-Serine
left: Homocysteine
name: Homocysteine + L-Serine → L-Cystathionine + Water
conversion direction: LEFT_TO_RIGHT

3-Phosphoglyceric acid
type: SmallMoleculeReference
xref: Reference/BioCyc G3P
xref: Reference/PubChem-compound 724
xref: Reference/ChEBI CHEBI 17050
xref: Reference/ChemSpider 704
xref: Reference/CAS 820-11-1
xref: Reference/KEGG Compound C00597
xref: Reference/HMDB HMDB0000807
chemical formula: C3H7O7P
molecular weight: 185.99294
structure: ChemicalStructure/PW C000644

Reference/HMDB HMDB0001487
type: UnificationXref
id: HMDB0001487
db: HMDB

ChemicalStructure/PW C000396
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CCCNC(N)=N)C(O)=O

Reference/BioCyc GLYOX
type: UnificationXref
id: GLYOX
db: BioCyc

Reference/KEGG Compound C00062
type: UnificationXref
id: C00062
db: KEGG Compound

Pyruvic acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Pyruvic acid

Compound/PW C001099 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Coenzyme A
stoichiometric coefficient: 1.0

5-Aminolevulinic acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 137
xref: Reference/BioCyc 5-AMINO-LEVULINATE
xref: Reference/ChEBI CHEBI 17549
xref: Reference/ChemSpider 134
xref: Reference/HMDB HMDB0001149
xref: Reference/KEGG Compound C00430
xref: Reference/CAS 106-60-5
chemical formula: C5H9NO3
molecular weight: 131.05824
structure: ChemicalStructure/PW C000894

5,10-Methylene-THF
type: SmallMolecule
cellular location: Cytosol
entity reference: 5,10-Methylene-THF

Reference/ChEBI CHEBI 29156
type: UnificationXref
id: CHEBI:29156
db: ChEBI

Reference/ChemSpider 5907
type: UnificationXref
id: 5907
db: ChemSpider

Compound/PW C001120 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: (R)-lipoic acid
stoichiometric coefficient: 1.0

Adenosine triphosphate
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 15422
xref: Reference/PubChem-compound 5957
xref: Reference/HMDB HMDB0000538
xref: Reference/CAS 56-65-5
xref: Reference/ChemSpider 5742
xref: Reference/BioCyc ATP
xref: Reference/KEGG Compound C00002
chemical formula: C10H16N5O13P3
molecular weight: 506.99576
structure: ChemicalStructure/PW C000414

Reference/KEGG Compound C00067
type: UnificationXref
id: C00067
db: KEGG Compound

Reference/KEGG Compound C00065
type: UnificationXref
id: C00065
db: KEGG Compound

Compound/PW C000120 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Serine
stoichiometric coefficient: 1.0

NAD
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: NAD

Pyrophosphate
type: SmallMolecule
cellular location: Cytoplasm
entity reference: Pyrophosphate

ChemicalStructure/PW C000164
type: ChemicalStructure
structure format: SMILES
structure data: CC(=O)C(O)=O

ReactionCatalysis1513
type: Catalysis
controller: Serine--pyruvate aminotransferase
controlled: Reaction1769
control type: ACTIVATION

ReactionCatalysis1515
type: Catalysis
controller: Glycine amidinotransferase, mitochondrial
controlled: 2.1.4.1
control type: ACTIVATION

Reference/KEGG Compound C00073
type: UnificationXref
id: C00073
db: KEGG Compound

ReactionCatalysis1514
type: Catalysis
controller: Serine--pyruvate aminotransferase
controlled: Reaction1770
control type: ACTIVATION

ReactionCatalysis1516
type: Catalysis
controller: Guanidinoacetate N-methyltransferase
controlled: 2.1.1.2
control type: ACTIVATION

Reference/HMDB HMDB0000161
type: UnificationXref
id: HMDB0000161
db: HMDB

Reference/UniProt P50440
type: UnificationXref
id: P50440
db: UniProt

3-Phosphoglyceric acid
type: SmallMolecule
cellular location: Cytosol
entity reference: 3-Phosphoglyceric acid
name: SubPathwayOutput

Reference/PubChem-compound 962
type: UnificationXref
id: 962
db: PubChem-compound

Reference/BioCyc DIMETHYL-GLYCINE
type: UnificationXref
id: DIMETHYL-GLYCINE
db: BioCyc

Reference/ChemSpider 31983
type: UnificationXref
id: 31983
db: ChemSpider

Reference/PubChem-compound 724
type: UnificationXref
id: 724
db: PubChem-compound

Reference/PubChem-compound 964
type: UnificationXref
id: 964
db: PubChem-compound

Serine--pyruvate aminotransferase
type: Complex
component stoichiometry: Protein/734 Mitochondrial Matrix/Stoichiometry/2.0
cellular location: Mitochondrial Matrix
component: Serine--pyruvate aminotransferase

Carbon dioxide
type: SmallMoleculeReference
xref: Reference/PubChem-compound 280
xref: Reference/ChEBI CHEBI 16526
xref: Reference/ChemSpider 274
xref: Reference/HMDB HMDB0001967
xref: Reference/CAS 124-38-9
xref: Reference/KEGG Compound C00011
chemical formula: CO2
molecular weight: 43.98983
structure: ChemicalStructure/PW C001316

Reference/ChemSpider 857
type: UnificationXref
id: 857
db: ChemSpider

Reference/HMDB HMDB0001024
type: UnificationXref
id: HMDB0001024
db: HMDB

ChemicalStructure/PW C001065
type: ChemicalStructure
structure format: SMILES
structure data: O=O

Reference/BioCyc BETAINE
type: UnificationXref
id: BETAINE
db: BioCyc

Glycine dehydrogenase [decarboxylating], mitochondrial
type: ProteinReference
xref: Reference/UniProt P23378
organism: Homo sapiens

Reference/HMDB HMDB0002111
type: UnificationXref
id: HMDB0002111
db: HMDB

Reference/HMDB HMDB0001022
type: UnificationXref
id: HMDB0001022
db: HMDB

SubPathwayInteraction621
type: Interaction
name: SubPathway621Reaction
name: SubPathwayReaction
participant: Pyruvic acid

Glycerate kinase
type: ProteinReference
xref: Reference/UniProt Q8IVS8
organism: Homo sapiens

Compound/PW C001102 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Formaldehyde
stoichiometric coefficient: 1.0

Reference/CAS 14265-44-2
type: UnificationXref
id: 14265-44-2
db: CAS

SubPathwayInteraction624
type: Interaction
name: SubPathwayReaction
name: SubPathway624Reaction
participant: 3-Phosphoglyceric acid

Reference/BioCyc PYRUVATE
type: UnificationXref
id: PYRUVATE
db: BioCyc

Reference/PubChem-compound 712
type: UnificationXref
id: 712
db: PubChem-compound

Reference/ChEBI CHEBI 15811
type: UnificationXref
id: CHEBI:15811
db: ChEBI

SubPathwayInteraction623
type: Interaction
name: SubPathwayReaction
name: SubPathway623Reaction
participant: 3-Phosphoglyceric acid

SubPathwayInteraction629
type: Interaction
name: SubPathway629Reaction
name: SubPathwayReaction
participant: L-Serine

Cystathionine gamma-lyase
type: ProteinReference
xref: Reference/UniProt P32929
organism: Homo sapiens

Reference/KEGG Compound C00091
type: UnificationXref
id: C00091
db: KEGG Compound

Reference/BioCyc G3P
type: UnificationXref
id: G3P
db: BioCyc

2-Ketobutyric acid
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 30831
xref: Reference/HMDB HMDB0000005
xref: Reference/CAS 600-18-0
xref: Reference/ChemSpider 57
xref: Reference/KEGG Compound C00109
xref: Reference/PubChem-compound 58
xref: Reference/BioCyc 2-OXOBUTANOATE
chemical formula: C4H6O3
molecular weight: 102.03169
structure: ChemicalStructure/PW C000003

Reference/HMDB HMDB0000187
type: UnificationXref
id: HMDB0000187
db: HMDB

Compound/PW C000003 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-Ketobutyric acid
stoichiometric coefficient: 1.0

Reference/PubChem-compound 644102
type: UnificationXref
id: 644102
db: PubChem-compound

L-Cysteine
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000574
xref: Reference/CAS 52-90-4
xref: Reference/KEGG Compound C00097
xref: Reference/BioCyc CYS
xref: Reference/ChemSpider 5653
xref: Reference/PubChem-compound 5862
xref: Reference/ChEBI CHEBI 17561
chemical formula: C3H7NO2S
molecular weight: 121.01975
structure: ChemicalStructure/PW C000448

Reference/KEGG Compound C00097
type: UnificationXref
id: C00097
db: KEGG Compound

Compound/PW C001316 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Carbon dioxide
stoichiometric coefficient: 1.0

Compound/PW C001221 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Tetrahydrofolic acid
stoichiometric coefficient: 1.0

ChemicalStructure/PW C001043
type: ChemicalStructure
structure format: SMILES
structure data: OCC(=O)C(O)=O

Hydrogen peroxide
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0003125
xref: Reference/PubChem-compound 784
xref: Reference/ChemSpider 763
xref: Reference/CAS 7722-84-1
xref: Reference/BioCyc HYDROGEN-PEROXIDE
xref: Reference/ChEBI CHEBI 16240
xref: Reference/KEGG Compound C00027
chemical formula: H2O2
molecular weight: 34.005478
structure: ChemicalStructure/PW C001783

Reference/BioCyc 6-S-ACETYL-DIHYDROLIPOATE
type: UnificationXref
id: 6-S-ACETYL-DIHYDROLIPOATE
db: BioCyc

Glycine
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Glycine

Reference/BioCyc NADH
type: UnificationXref
id: NADH
db: BioCyc

Reference/HMDB HMDB0002134
type: UnificationXref
id: HMDB0002134
db: HMDB

Reference/BioCyc CREATINE
type: UnificationXref
id: CREATINE
db: BioCyc

Pyruvaldehyde
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Pyruvaldehyde

Compound/PW C000548 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Methionine
stoichiometric coefficient: 1.0

Reference/PubChem-compound 977
type: UnificationXref
id: 977
db: PubChem-compound

Sarcosine dehydrogenase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Sarcosine dehydrogenase, mitochondrial

Compound/PW C001221 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Tetrahydrofolic acid
stoichiometric coefficient: 1.0

Reference/CAS 50-00-0
type: UnificationXref
id: 50-00-0
db: CAS

Compound/PW C001104 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Phosphate
stoichiometric coefficient: 1.0

Glycine--tRNA ligase
type: ProteinReference
xref: Reference/UniProt P41250
organism: Homo sapiens

Acetyl-CoA
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Acetyl-CoA

Reference/KEGG Compound C02291
type: UnificationXref
id: C02291
db: KEGG Compound

Reference/CAS 820-11-1
type: UnificationXref
id: 820-11-1
db: CAS

Compound/PW C001875 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: D-Serine
stoichiometric coefficient: 1.0

Reference/ChemSpider 30572
type: UnificationXref
id: 30572
db: ChemSpider

ChemicalStructure/PW C000448
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CS)C(O)=O

Reference/KEGG Compound C05330
type: UnificationXref
id: C05330
db: KEGG Compound

(R)-lipoic acid
type: SmallMoleculeReference
xref: Reference/KEGG Compound C16241
xref: Reference/ChEBI CHEBI 30314
xref: Reference/CAS 62-46-4
xref: Reference/PubChem-compound 6112
xref: Reference/ChemSpider 5886
xref: Reference/BioCyc LIPOIC-ACID
xref: Reference/HMDB HMDB0001451
chemical formula: C8H14O2S2
molecular weight: 206.04352
structure: ChemicalStructure/PW C001120

ChemicalStructure/PW C001783
type: ChemicalStructure
structure format: SMILES
structure data: OO

Ammonia
type: SmallMoleculeReference
xref: Reference/BioCyc AMMONIA
xref: Reference/CAS 7664-41-7
xref: Reference/HMDB HMDB0000051
xref: Reference/PubChem-compound 222
xref: Reference/KEGG Compound C00014
xref: Reference/ChEBI CHEBI 16134
xref: Reference/ChemSpider 217
chemical formula: H3N
molecular weight: 17.026548
structure: ChemicalStructure/PW C000035

Compound/PW C000030 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: βine
stoichiometric coefficient: 1.0

Adenosine monophosphate
type: SmallMoleculeReference
xref: Reference/CAS 61-19-8
xref: Reference/ChemSpider 5858
xref: Reference/HMDB HMDB0000045
xref: Reference/BioCyc AMP
xref: Reference/ChEBI CHEBI 16027
xref: Reference/PubChem-compound 6083
xref: Reference/KEGG Compound C00020
chemical formula: C10H14N5O7P
molecular weight: 347.06308
structure: ChemicalStructure/PW C000032

βine
type: SmallMoleculeReference
xref: Reference/PubChem-compound 247
xref: Reference/BioCyc BETAINE
xref: Reference/ChemSpider 242
xref: Reference/KEGG Compound C00719
xref: Reference/CAS 107-43-7
xref: Reference/ChEBI CHEBI 17750
xref: Reference/HMDB HMDB0000043
chemical formula: C5H11NO2
molecular weight: 117.07898
structure: ChemicalStructure/PW C000030

Protein/289 Mitochondrial Matrix/Stoichiometry/4.0
type: Stoichiometry
physical entity: Aldehyde dehydrogenase, mitochondrial
stoichiometric coefficient: 4.0

Compound/PW C001065 Mitochondrial Outer Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Oxygen
stoichiometric coefficient: 1.0

Compound/PW C007757 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Dihydrolipoate
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0000538
type: UnificationXref
id: HMDB0000538
db: HMDB

Compound/PW C000067 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Cystathionine
stoichiometric coefficient: 1.0

Phosphoserine phosphatase
type: ProteinReference
xref: Reference/UniProt P78330
organism: Homo sapiens

Reference/BioCyc Serines
type: UnificationXref
id: Serines
db: BioCyc

Reference/PubChem-compound 6816
type: UnificationXref
id: 6816
db: PubChem-compound

Compound/PW C000185 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Sarcosine
stoichiometric coefficient: 1.0

Reference/PubChem-compound 51
type: UnificationXref
id: 51
db: PubChem-compound

Reference/ChemSpider 242
type: UnificationXref
id: 242
db: ChemSpider

Reference/ChEBI CHEBI 17906
type: UnificationXref
id: CHEBI:17906
db: ChEBI

Creatine
type: SmallMoleculeReference
xref: Reference/ChemSpider 566
xref: Reference/PubChem-compound 586
xref: Reference/CAS 57-00-1
xref: Reference/HMDB HMDB0000064
xref: Reference/KEGG Compound C00300
xref: Reference/BioCyc CREATINE
xref: Reference/ChEBI CHEBI 16919
chemical formula: C4H9N3O2
molecular weight: 131.06947
structure: ChemicalStructure/PW C000046

Reference/PubChem-compound 58
type: UnificationXref
id: 58
db: PubChem-compound

Reference/PubChem-compound 49791978
type: UnificationXref
id: 49791978
db: PubChem-compound

L-Cystathionine
type: SmallMolecule
cellular location: Cytosol
entity reference: L-Cystathionine

Reference/KEGG Compound C16241
type: UnificationXref
id: C16241
db: KEGG Compound

Formaldehyde
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 16842
xref: Reference/BioCyc FORMALDEHYDE
xref: Reference/HMDB HMDB0001426
xref: Reference/PubChem-compound 712
xref: Reference/ChemSpider 692
xref: Reference/KEGG Compound C00067
xref: Reference/CAS 50-00-0
chemical formula: CH2O
molecular weight: 30.010565
structure: ChemicalStructure/PW C001102

Reference/ChemSpider 274
type: UnificationXref
id: 274
db: ChemSpider

Reference/UniProt P23378
type: UnificationXref
id: P23378
db: UniProt

Reference/PubChem-compound 137
type: UnificationXref
id: 137
db: PubChem-compound

Reference/ChemSpider 141140
type: UnificationXref
id: 141140
db: ChemSpider

Reference/UniProt Q14353
type: UnificationXref
id: Q14353
db: UniProt

Reference/CAS 485-80-3
type: UnificationXref
id: 485-80-3
db: CAS

Phosphate
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00009
xref: Reference/PubChem-compound 1061
xref: Reference/CAS 14265-44-2
xref: Reference/HMDB HMDB0001429
xref: Reference/BioCyc CPD-8587
xref: Reference/ChEBI CHEBI 18367
xref: Reference/ChemSpider 1032
chemical formula: H3O4P
molecular weight: 97.9769
structure: ChemicalStructure/PW C001104

L-2-Amino-3-oxobutanoic acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: L-2-Amino-3-oxobutanoic acid

Reference/BioCyc SER
type: UnificationXref
id: SER
db: BioCyc

Compound/PW C000144 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Orotidylic acid
stoichiometric coefficient: 1.0

Serine--tRNA ligase, cytoplasmic
type: ProteinReference
xref: Reference/UniProt P49591
organism: Homo sapiens

SubPathwayInteraction609
type: Interaction
name: SubPathwayReaction
name: SubPathway609Reaction
participant: L-Cysteine

Reference/UniProt Q9Y617
type: UnificationXref
id: Q9Y617
db: UniProt

Amine oxidase [flavin-containing] A
type: Protein
cellular location: Mitochondrial Outer Membrane
entity reference: Amine oxidase [flavin-containing] A

Reference/BioCyc AMINO-ACETONE
type: UnificationXref
id: AMINO-ACETONE
db: BioCyc

RNA/27 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Seryl-tRNA
stoichiometric coefficient: 1.0

Reference/BioCyc CPD-6641
type: UnificationXref
id: CPD-6641
db: BioCyc

L-Seryl-tRNA
type: Rna
cellular location: Cytoplasm
entity reference: L-Seryl-tRNA

Reference/ChemSpider 217
type: UnificationXref
id: 217
db: ChemSpider

Reference/CAS 58-68-4
type: UnificationXref
id: 58-68-4
db: CAS

Reference/HMDB HMDB0000574
type: UnificationXref
id: HMDB0000574
db: HMDB

Compound/PW C001420 Mitochondrial Outer Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Water
stoichiometric coefficient: 1.0

Dimethylglycine dehydrogenase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Dimethylglycine dehydrogenase, mitochondrial

Reference/SMPDB SMP0000040
type: UnificationXref
id: SMP0000040
db: SMPDB

Reference/HMDB HMDB0001423
type: UnificationXref
id: HMDB0001423
db: HMDB

ChemicalStructure/PW C001104
type: ChemicalStructure
structure format: SMILES
structure data: OP(O)(O)=O

Reference/ChemSpider 210
type: UnificationXref
id: 210
db: ChemSpider

(R)-lipoic acid
type: SmallMolecule
cellular location: Mitochondrion
entity reference: (R)-lipoic acid

ChemicalStructure/PW C001102
type: ChemicalStructure
structure format: SMILES
structure data: C=O

Reference/HMDB HMDB0001426
type: UnificationXref
id: HMDB0001426
db: HMDB

Reference/ChemSpider 692
type: UnificationXref
id: 692
db: ChemSpider

Reference/ChEBI CHEBI 30915
type: UnificationXref
id: CHEBI:30915
db: ChEBI

Reference/HMDB HMDB0013639
type: UnificationXref
id: HMDB0013639
db: HMDB

Glycine
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000123
xref: Reference/BioCyc GLY
xref: Reference/ChemSpider 730
xref: Reference/PubChem-compound 5257127
xref: Reference/CAS 56-40-6
xref: Reference/ChEBI CHEBI 15428
xref: Reference/KEGG Compound C00037
chemical formula: C2H5NO2
molecular weight: 75.03203
structure: ChemicalStructure/PW C000078

Glyoxylic acid
type: SmallMoleculeReference
xref: Reference/BioCyc GLYOX
xref: Reference/ChemSpider 740
xref: Reference/CAS 298-12-4
xref: Reference/HMDB HMDB0000119
xref: Reference/PubChem-compound 760
xref: Reference/ChEBI CHEBI 16891
xref: Reference/KEGG Compound C00048
chemical formula: C2H2O3
molecular weight: 74.0004
structure: ChemicalStructure/PW C000075

Reference/CAS 298-08-8
type: UnificationXref
id: 298-08-8
db: CAS

Protein/536 Cytoplasm/Stoichiometry/2.0
type: Stoichiometry
physical entity: Glycine--tRNA ligase
stoichiometric coefficient: 2.0

Reference/HMDB HMDB0001429
type: UnificationXref
id: HMDB0001429
db: HMDB

Guanidoacetic acid
type: SmallMoleculeReference
xref: Reference/ChemSpider 743
xref: Reference/ChEBI CHEBI 16344
xref: Reference/BioCyc GUANIDOACETIC ACID
xref: Reference/CAS 352-97-6
xref: Reference/HMDB HMDB0000128
xref: Reference/KEGG Compound C00581
xref: Reference/PubChem-compound 3946848
chemical formula: C3H7N3O2
molecular weight: 117.053825
structure: ChemicalStructure/PW C000083

Compound/PW C000090 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Glyceric acid
stoichiometric coefficient: 1.0

Reference/GENE ONTOLOGY GO 0005759
type: UnificationXref
id: GO:0005759
db: GENE ONTOLOGY

5,10-Methylene-THF
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001533
xref: Reference/CAS 3432-99-3
xref: Reference/PubChem-compound 439175
xref: Reference/ChEBI CHEBI 15636
xref: Reference/KEGG Compound C00143
xref: Reference/BioCyc METHYLENE-THF
xref: Reference/ChemSpider 388320
chemical formula: C20H23N7O6
molecular weight: 457.171
structure: ChemicalStructure/PW C001178

Compound/PW C000164 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyruvic acid
stoichiometric coefficient: 1.0

Dimethylglycine
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Dimethylglycine

Gluconeogenesis
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway623
pathway component: SubPathwayInteraction623
xref: Reference/SMPDB SMP0000128
xref: Reference/PathWhiz PW000152
name: SubPathway

Reference/PubChem-compound 105
type: UnificationXref
id: 105
db: PubChem-compound

Reference/PubChem-compound 586
type: UnificationXref
id: 586
db: PubChem-compound

Reference/UniProt O43175
type: UnificationXref
id: O43175
db: UniProt

Oxoglutaric acid
type: SmallMolecule
cellular location: Cytosol
entity reference: Oxoglutaric acid

ChemicalStructure/PW C001316
type: ChemicalStructure
structure format: SMILES
structure data: O=C=O

Reference/PubChem-compound 5950
type: UnificationXref
id: 5950
db: PubChem-compound

Reference/PubChem-compound 5951
type: UnificationXref
id: 5951
db: PubChem-compound

Homocysteine
type: SmallMolecule
cellular location: Cytosol
entity reference: Homocysteine

Protein/740 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: Glycine dehydrogenase [decarboxylating], mitochondrial
stoichiometric coefficient: 2.0

Reference/SMPDB SMP0000060
type: UnificationXref
id: SMP0000060
db: SMPDB

NADH
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001487
xref: Reference/CAS 58-68-4
xref: Reference/PubChem-compound 439153
xref: Reference/BioCyc NADH
xref: Reference/ChemSpider 388299
xref: Reference/KEGG Compound C00004
xref: Reference/ChEBI CHEBI 16908
chemical formula: C21H29N7O14P2
molecular weight: 665.12476
structure: ChemicalStructure/PW C001144

Protein/734 Mitochondrial Matrix/Stoichiometry/2.0
type: Stoichiometry
physical entity: Serine--pyruvate aminotransferase
stoichiometric coefficient: 2.0

Reference/UniProt P21397
type: UnificationXref
id: P21397
db: UniProt

Glycine amidinotransferase, mitochondrial
type: Complex
component stoichiometry: Protein/447 Mitochondrion/Stoichiometry/2.0
cellular location: Mitochondrion
component: Glycine amidinotransferase, mitochondrial

Reference/HMDB HMDB0001206
type: UnificationXref
id: HMDB0001206
db: HMDB

Reference/PubChem-compound 5957
type: UnificationXref
id: 5957
db: PubChem-compound

Reference/GENE ONTOLOGY GO 0005743
type: UnificationXref
id: GO:0005743
db: GENE ONTOLOGY

Reference/GENE ONTOLOGY GO 0005741
type: UnificationXref
id: GO:0005741
db: GENE ONTOLOGY

Reference/ChEBI CHEBI 16842
type: UnificationXref
id: CHEBI:16842
db: ChEBI

Reference/HMDB HMDB0000119
type: UnificationXref
id: HMDB0000119
db: HMDB

Reference/BioCyc 3-P-HYDROXYPYRUVATE
type: UnificationXref
id: 3-P-HYDROXYPYRUVATE
db: BioCyc

Reference/HMDB HMDB0000123
type: UnificationXref
id: HMDB0000123
db: HMDB

Reference/GENE ONTOLOGY GO 0005739
type: UnificationXref
id: GO:0005739
db: GENE ONTOLOGY

Compound/PW C000808 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Succinyl-CoA
stoichiometric coefficient: 1.0

Reference/GENE ONTOLOGY GO 0005737
type: UnificationXref
id: GO:0005737
db: GENE ONTOLOGY

ChemicalStructure/PW C000003
type: ChemicalStructure
structure format: SMILES
structure data: CCC(=O)C(O)=O

Reference/HMDB HMDB0000128
type: UnificationXref
id: HMDB0000128
db: HMDB

L-Cystathionine
type: SmallMoleculeReference
xref: Reference/KEGG Compound C02291
xref: Reference/BioCyc L-CYSTATHIONINE
xref: Reference/PubChem-compound 25243997
xref: Reference/ChEBI CHEBI 17482
xref: Reference/ChemSpider 388392
xref: Reference/CAS 56-88-2
xref: Reference/HMDB HMDB0000099
chemical formula: C7H14N2O4S
molecular weight: 222.06743
structure: ChemicalStructure/PW C000067

Reference/HMDB HMDB0001451
type: UnificationXref
id: HMDB0001451
db: HMDB

Dimethylglycine
type: SmallMoleculeReference
xref: Reference/BioCyc DIMETHYL-GLYCINE
xref: Reference/ChemSpider 653
xref: Reference/CAS 1118-68-9
xref: Reference/ChEBI CHEBI 17724
xref: Reference/HMDB HMDB0000092
xref: Reference/PubChem-compound 673
xref: Reference/KEGG Compound C01026
chemical formula: C4H9NO2
molecular weight: 103.06333
structure: ChemicalStructure/PW C000062

Reference/BioCyc OROTIDINE-5-PHOSPHATE
type: UnificationXref
id: OROTIDINE-5-PHOSPHATE
db: BioCyc

Reference/ChEBI CHEBI 16891
type: UnificationXref
id: CHEBI:16891
db: ChEBI

Reference/ChemSpider 389046
type: UnificationXref
id: 389046
db: ChemSpider

Reference/ChemSpider 5682
type: UnificationXref
id: 5682
db: ChemSpider

5,10-Methylene-THF
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 5,10-Methylene-THF

Reference/BioCyc METHYL-GLYOXAL
type: UnificationXref
id: METHYL-GLYOXAL
db: BioCyc

1.4.3.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Mitochondrial Outer Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000035 Mitochondrial Outer Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001065 Mitochondrial Outer Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001435 Mitochondrial Outer Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000905 Mitochondrial Outer Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001783 Mitochondrial Outer Membrane/Stoichiometry/1.0
right: Ammonia
right: Hydrogen peroxide
right: Pyruvaldehyde
spontaneous: false
EC number: 1.4.3.4
left: Oxygen
left: Water
left: Aminoacetone
name: Aminoacetone + Oxygen + Water → Ammonia + Hydrogen peroxide + Pyruvaldehyde
conversion direction: LEFT_TO_RIGHT

Reference/KEGG Compound C00719
type: UnificationXref
id: C00719
db: KEGG Compound

Glyoxylic acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Glyoxylic acid

Mitochondrial Matrix
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005759

Formaldehyde
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Formaldehyde

Compound/PW C000448 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Cysteine
stoichiometric coefficient: 1.0

Reference/CAS 14000-31-8
type: UnificationXref
id: 14000-31-8
db: CAS

L-2-Amino-3-oxobutanoic acid
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: L-2-Amino-3-oxobutanoic acid

Reference/BioCyc CO-A
type: UnificationXref
id: CO-A
db: BioCyc

Reference/UniProt P05091
type: UnificationXref
id: P05091
db: UniProt

Guanidinoacetate N-methyltransferase
type: Protein
cellular location: Cytosol
entity reference: Guanidinoacetate N-methyltransferase

Reference/CAS 72-89-9
type: UnificationXref
id: 72-89-9
db: CAS

Reference/ChEBI CHEBI 17750
type: UnificationXref
id: CHEBI:17750
db: ChEBI

Reference/HMDB HMDB0000939
type: UnificationXref
id: HMDB0000939
db: HMDB

Compound/PW C000083 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Guanidoacetic acid
stoichiometric coefficient: 1.0

Dihydrolipoate
type: SmallMoleculeReference
xref: Reference/PubChem-compound 421
xref: Reference/CAS 462-20-4
xref: Reference/BioCyc 6-S-ACETYL-DIHYDROLIPOATE
xref: Reference/KEGG Compound C02147
xref: Reference/ChemSpider 408
xref: Reference/ChEBI CHEBI 18047
xref: Reference/HMDB HMDB0012210
chemical formula: C8H16O2S2
molecular weight: 208.05917
structure: ChemicalStructure/PW C007757

Compound/PW C001043 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Hydroxypyruvic acid
stoichiometric coefficient: 1.0

Reference/SMPDB SMP0000013
type: UnificationXref
id: SMP0000013
db: SMPDB

Reference/UniProt Q8IVS8
type: UnificationXref
id: Q8IVS8
db: UniProt

Reference/PathWhiz PW000209
type: UnificationXref
id: PW000209
db: PathWhiz

Reference/ChemSpider 6557
type: UnificationXref
id: 6557
db: ChemSpider

Reference/ChEBI CHEBI 17724
type: UnificationXref
id: CHEBI:17724
db: ChEBI

Reference/CAS 106-60-5
type: UnificationXref
id: 106-60-5
db: CAS

2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial
type: ProteinReference
xref: Reference/UniProt O75600
organism: Homo sapiens

2.3.1.29
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000940 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000078 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C002747 Mitochondrial Matrix/Stoichiometry/1.0
right: Coenzyme A
right: L-2-Amino-3-oxobutanoic acid
spontaneous: false
EC number: 2.3.1.29
left: Acetyl-CoA
left: Glycine
name: Acetyl-CoA + Glycine → Coenzyme A + L-2-Amino-3-oxobutanoic acid
conversion direction: LEFT_TO_RIGHT

Reference/CAS 127-17-3
type: UnificationXref
id: 127-17-3
db: CAS

Glyceric acid
type: SmallMoleculeReference
xref: Reference/ChemSpider 388334
xref: Reference/HMDB HMDB0000139
xref: Reference/PubChem-compound 439194
xref: Reference/ChEBI CHEBI 32398
xref: Reference/KEGG Compound C00258
xref: Reference/BioCyc 2-PG
xref: Reference/CAS 473-81-4
chemical formula: C3H6O4
molecular weight: 106.02661
structure: ChemicalStructure/PW C000090

Reference/ChEBI CHEBI 30933
type: UnificationXref
id: CHEBI:30933
db: ChEBI

Reference/PubChem-compound 28782
type: UnificationXref
id: 28782
db: PubChem-compound

Hydrogen peroxide
type: SmallMolecule
cellular location: Mitochondrial Outer Membrane
entity reference: Hydrogen peroxide

1.4.4.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001120 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C008526 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000078 Mitochondrial Matrix/Stoichiometry/1.0
right: Carbon dioxide
right: 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid
spontaneous: false
EC number: 1.4.4.2
left: Glycine
left: (R)-lipoic acid
name: (R)-lipoic acid + Glycine → 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid + Carbon dioxide
conversion direction: LEFT_TO_RIGHT

Reference/CAS 312-84-5
type: UnificationXref
id: 312-84-5
db: CAS

Reference/UniProt Q14749
type: UnificationXref
id: Q14749
db: UniProt

1.5.8.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001102 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000185 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000078 Mitochondrial Matrix/Stoichiometry/1.0
right: Glycine
right: Formaldehyde
spontaneous: false
EC number: 1.5.8.3
left: Water
left: Sarcosine
name: Sarcosine + Water → Formaldehyde + Glycine
conversion direction: LEFT_TO_RIGHT

L-Glutamic acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 33032
xref: Reference/ChEBI CHEBI 16015
xref: Reference/ChemSpider 30572
xref: Reference/HMDB HMDB0000148
xref: Reference/CAS 56-86-0
xref: Reference/BioCyc GLT
xref: Reference/KEGG Compound C00025
chemical formula: C5H9NO4
molecular weight: 147.05316
structure: ChemicalStructure/PW C000095

Water
type: SmallMolecule
cellular location: Cytosol
entity reference: Water

1.5.8.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000062 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001102 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000185 Mitochondrial Matrix/Stoichiometry/1.0
right: Formaldehyde
right: Sarcosine
spontaneous: false
EC number: 1.5.8.4
left: Water
left: Dimethylglycine
name: Dimethylglycine + Water → Formaldehyde + Sarcosine
conversion direction: LEFT_TO_RIGHT

Reference/PubChem-compound 33032
type: UnificationXref
id: 33032
db: PubChem-compound

Reference/ChEBI CHEBI 16643
type: UnificationXref
id: CHEBI:16643
db: ChEBI

Compound/PW C007757 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Dihydrolipoate
stoichiometric coefficient: 1.0

Dihydrolipoyl dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P09622
organism: Homo sapiens

Reference/UniProt P34897
type: UnificationXref
id: P34897
db: UniProt

Reaction1429
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002747 Mitochondrial Inner Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Mitochondrial Inner Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001435 Mitochondrial Inner Membrane/Stoichiometry/1.0
right: Carbon dioxide
right: Aminoacetone
spontaneous: false
left: L-2-Amino-3-oxobutanoic acid
name: L-2-Amino-3-oxobutanoic acid → Aminoacetone + Carbon dioxide
conversion direction: LEFT_TO_RIGHT

Reference/UniProt P34896
type: UnificationXref
id: P34896
db: UniProt

Phosphoserine
type: SmallMolecule
cellular location: Cytosol
entity reference: Phosphoserine

Reference/KEGG Compound C00740
type: UnificationXref
id: C00740
db: KEGG Compound

Reference/CAS 7782-44-7
type: UnificationXref
id: 7782-44-7
db: CAS

Compound/PW C000396 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Arginine
stoichiometric coefficient: 1.0

1.2.1.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000164 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000905 Mitochondrial Matrix/Stoichiometry/1.0
right: Water
right: Pyruvic acid
right: NADH
spontaneous: false
EC number: 1.2.1.3
left: NAD
left: Pyruvaldehyde
name: NAD + Pyruvaldehyde → NADH + Pyruvic acid + Water
conversion direction: LEFT_TO_RIGHT

Reference/SMPDB SMP0000034
type: UnificationXref
id: SMP0000034
db: SMPDB

NADH
type: SmallMolecule
cellular location: Mitochondrion
entity reference: NADH

Reference/TAXONOMY 9606
type: UnificationXref
id: 9606
db: TAXONOMY

Reference/ChEBI CHEBI 17549
type: UnificationXref
id: CHEBI:17549
db: ChEBI

Reference/BioCyc 2-KETOGLUTARATE
type: UnificationXref
id: 2-KETOGLUTARATE
db: BioCyc

Reference/ChemSpider 392413
type: UnificationXref
id: 392413
db: ChemSpider

ChemicalStructure/PW C000644
type: ChemicalStructure
structure format: SMILES
structure data: OC(COP(O)(O)=O)C(O)=O

Reference/ChemSpider 388392
type: UnificationXref
id: 388392
db: ChemSpider

L-Methionine
type: SmallMolecule
cellular location: Cytosol
entity reference: L-Methionine

Pyruvaldehyde
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001167
xref: Reference/KEGG Compound C00546
xref: Reference/ChEBI CHEBI 17158
xref: Reference/CAS 78-98-8
xref: Reference/BioCyc METHYL-GLYOXAL
xref: Reference/ChemSpider 857
xref: Reference/PubChem-compound 880
chemical formula: C3H4O2
molecular weight: 72.021126
structure: ChemicalStructure/PW C000905

ChemicalStructure/PW C000894
type: ChemicalStructure
structure format: SMILES
structure data: NCC(=O)CCC(O)=O

L-Seryl-tRNA(Ser)
type: Rna
cellular location: Cytoplasm
entity reference: L-Seryl-tRNA(Ser)

Reference/PathWhiz PW000018
type: UnificationXref
id: PW000018
db: PathWhiz

2.1.2.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001420 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001178 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001221 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000078 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000120 Mitochondrial Matrix/Stoichiometry/1.0
right: L-Serine
right: Tetrahydrofolic acid
spontaneous: false
EC number: 2.1.2.1
left: 5,10-Methylene-THF
left: Glycine
left: Water
name: 5,10-Methylene-THF + Glycine + Water ↔ L-Serine + Tetrahydrofolic acid
conversion direction: REVERSIBLE

Reference/ChEBI CHEBI 15377
type: UnificationXref
id: CHEBI:15377
db: ChEBI

L-Cysteine
type: SmallMolecule
cellular location: Cytosol
entity reference: L-Cysteine
name: SubPathwayInput

Reference/ChEBI CHEBI 15379
type: UnificationXref
id: CHEBI:15379
db: ChEBI

Reference/ChEBI CHEBI 16467
type: UnificationXref
id: CHEBI:16467
db: ChEBI

Reference/CAS 56-45-1
type: UnificationXref
id: 56-45-1
db: CAS

ChemicalStructure/PW C000414
type: ChemicalStructure
structure format: SMILES

Glycine dehydrogenase [decarboxylating], mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Glycine dehydrogenase [decarboxylating], mitochondrial

Compound/PW C000078 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Glycine
stoichiometric coefficient: 1.0

Serine hydroxymethyltransferase, cytosolic
type: Complex
component stoichiometry: Protein/739 Cytosol/Stoichiometry/4.0
cellular location: Cytosol
component: Serine hydroxymethyltransferase, cytosolic

Reference/HMDB HMDB0000742
type: UnificationXref
id: HMDB0000742
db: HMDB

Dimethylglycine dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q9UI17
organism: Homo sapiens

Reference/BioCyc AMINO-OXOBUT
type: UnificationXref
id: AMINO-OXOBUT
db: BioCyc

ReactionCatalysis1498
type: Catalysis
controller: Dihydrolipoyl dehydrogenase, mitochondrial
controlled: 1.8.1.4
control type: ACTIVATION

2.1.2.10
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001178 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C008526 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C007757 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001221 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000035 Mitochondrial Matrix/Stoichiometry/1.0
right: 5,10-Methylene-THF
right: Ammonia
right: Dihydrolipoate
spontaneous: false
EC number: 2.1.2.10
left: 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid
left: Tetrahydrofolic acid
name: 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid + Tetrahydrofolic acid → 5,10-Methylene-THF + Ammonia + Dihydrolipoate
conversion direction: LEFT_TO_RIGHT

5-aminolevulinate synthase, nonspecific, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: 5-aminolevulinate synthase, nonspecific, mitochondrial

Reference/UniProt O75600
type: UnificationXref
id: O75600
db: UniProt

Reference/SMPDB SMP0000223
type: UnificationXref
id: SMP0000223
db: SMPDB

Reference/BioCyc 3-METHYLBENZYLSUCCINYL-COA
type: UnificationXref
id: 3-METHYLBENZYLSUCCINYL-COA
db: BioCyc

Reference/KEGG Compound C00546
type: UnificationXref
id: C00546
db: KEGG Compound

Reference/CAS 74-79-3
type: UnificationXref
id: 74-79-3
db: CAS

Reference/HMDB HMDB0001846
type: UnificationXref
id: HMDB0001846
db: HMDB

Compound/PW C000164 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyruvic acid
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 16680
type: UnificationXref
id: CHEBI:16680
db: ChEBI

Reference/HMDB HMDB0000517
type: UnificationXref
id: HMDB0000517
db: HMDB

Reference/ChEBI CHEBI 15351
type: UnificationXref
id: CHEBI:15351
db: ChEBI

8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid

Reference/KEGG Compound C00300
type: UnificationXref
id: C00300
db: KEGG Compound

Reference/CAS 85-61-0
type: UnificationXref
id: 85-61-0
db: CAS

Compound/PW C000721 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: NAD
stoichiometric coefficient: 1.0

Dihydrolipoate
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Dihydrolipoate

Reference/UniProt P21549
type: UnificationXref
id: P21549
db: UniProt

Reference/CAS 454-29-5
type: UnificationXref
id: 454-29-5
db: CAS

Reference/KEGG Compound C01888
type: UnificationXref
id: C01888
db: KEGG Compound

Reference/CAS 7722-84-1
type: UnificationXref
id: 7722-84-1
db: CAS

Reference/PubChem-compound 6022
type: UnificationXref
id: 6022
db: PubChem-compound

Acetyl-CoA
type: SmallMoleculeReference
xref: Reference/BioCyc ACETYL-COA
xref: Reference/PubChem-compound 444493
xref: Reference/HMDB HMDB0001206
xref: Reference/ChemSpider 392413
xref: Reference/CAS 72-89-9
xref: Reference/ChEBI CHEBI 15351
xref: Reference/KEGG Compound C00024
chemical formula: C23H38N7O17P3S
molecular weight: 809.1258
structure: ChemicalStructure/PW C000940

Reference/ChemSpider 939
type: UnificationXref
id: 939
db: ChemSpider

L-Serine
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: L-Serine

Reference/ChemSpider 937
type: UnificationXref
id: 937
db: ChemSpider

Reference/BioCyc CYS
type: UnificationXref
id: CYS
db: BioCyc

Aminomethyltransferase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Aminomethyltransferase, mitochondrial

Reference/ChEBI CHEBI 16134
type: UnificationXref
id: CHEBI:16134
db: ChEBI

Reference/HMDB HMDB0003125
type: UnificationXref
id: HMDB0003125
db: HMDB

Reference/HMDB HMDB0000092
type: UnificationXref
id: HMDB0000092
db: HMDB

Reference/HMDB HMDB0001185
type: UnificationXref
id: HMDB0001185
db: HMDB

Reference/HMDB HMDB0000099
type: UnificationXref
id: HMDB0000099
db: HMDB

Reference/PathWhiz PW000054
type: UnificationXref
id: PW000054
db: PathWhiz

Reference/ChEBI CHEBI 17230
type: UnificationXref
id: CHEBI:17230
db: ChEBI

Reference/BioCyc ATP
type: UnificationXref
id: ATP
db: BioCyc

Reference/ChemSpider 18714427
type: UnificationXref
id: 18714427
db: ChemSpider

Protein/511 Cytosol/Stoichiometry/4.0
type: Stoichiometry
physical entity: Cystathionine gamma-lyase
stoichiometric coefficient: 4.0

Tetrahydrofolic acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Tetrahydrofolic acid

Glycine dehydrogenase [decarboxylating], mitochondrial
type: Complex
component stoichiometry: Protein/740 Mitochondrial Matrix/Stoichiometry/2.0
cellular location: Mitochondrial Matrix
component: Glycine dehydrogenase [decarboxylating], mitochondrial

Tetrahydrofolic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: Tetrahydrofolic acid

Reference/ChemSpider 388334
type: UnificationXref
id: 388334
db: ChemSpider

S-Adenosylmethionine
type: SmallMoleculeReference
xref: Reference/PubChem-compound 24762165
xref: Reference/KEGG Compound C00019
xref: Reference/ChemSpider 31983
xref: Reference/ChEBI CHEBI 15414
xref: Reference/CAS 485-80-3
xref: Reference/BioCyc S-ADENOSYLMETHIONINE
xref: Reference/HMDB HMDB0001185
chemical formula: C15H23N6O5S
molecular weight: 399.14505
structure: ChemicalStructure/PW C000921

Compound/PW C000921 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: S-Adenosylmethionine
stoichiometric coefficient: 1.0

Reference/ChemSpider 952
type: UnificationXref
id: 952
db: ChemSpider

Aldehyde dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P05091
organism: Homo sapiens

Reference/CAS 107-43-7
type: UnificationXref
id: 107-43-7
db: CAS

Reference/UniProt Q9UI17
type: UnificationXref
id: Q9UI17
db: UniProt

Reference/BioCyc ARG
type: UnificationXref
id: ARG
db: BioCyc

Reference/ChemSpider 5858
type: UnificationXref
id: 5858
db: ChemSpider

Adenosine monophosphate
type: SmallMolecule
cellular location: Cytoplasm
entity reference: Adenosine monophosphate

Carbon dioxide
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Carbon dioxide

Reference/KEGG Compound C00109
type: UnificationXref
id: C00109
db: KEGG Compound

Reference/BioCyc METHYLENE-THF
type: UnificationXref
id: METHYLENE-THF
db: BioCyc

Reference/KEGG Compound C00581
type: UnificationXref
id: C00581
db: KEGG Compound

Reference/ChemSpider 704
type: UnificationXref
id: 704
db: ChemSpider

Aminoacetone
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: Aminoacetone

Compound/PW C000078 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Glycine
stoichiometric coefficient: 1.0

Reference/KEGG Compound C00101
type: UnificationXref
id: C00101
db: KEGG Compound

ChemicalStructure/PW C007757
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CCCCC(S)CCS

Reference/PubChem-compound 440033
type: UnificationXref
id: 440033
db: PubChem-compound

Reference/BioCyc PPI
type: UnificationXref
id: PPI
db: BioCyc

Reference/PubChem-compound 160617
type: UnificationXref
id: 160617
db: PubChem-compound

5-aminolevulinate synthase, nonspecific, mitochondrial
type: ProteinReference
xref: Reference/UniProt P13196
organism: Homo sapiens

Reference/BioCyc FORMALDEHYDE
type: UnificationXref
id: FORMALDEHYDE
db: BioCyc

Compound/PW C000414 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Adenosine triphosphate
stoichiometric coefficient: 1.0

Reference/ChemSpider 5886
type: UnificationXref
id: 5886
db: ChemSpider

ChemicalStructure/PW C000921
type: ChemicalStructure
structure format: SMILES

Cytosol
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005829

Reference/KEGG Compound C00597
type: UnificationXref
id: C00597
db: KEGG Compound

S-Adenosylhomocysteine
type: SmallMoleculeReference
xref: Reference/PubChem-compound 25246222
xref: Reference/ChemSpider 388301
xref: Reference/BioCyc ADENOSYL-HOMO-CYS
xref: Reference/CAS 979-92-0
xref: Reference/KEGG Compound C00021
xref: Reference/ChEBI CHEBI 16680
xref: Reference/HMDB HMDB0000939
chemical formula: C14H20N6O5S
molecular weight: 384.12158
structure: ChemicalStructure/PW C000749

Reference/BioCyc SARCOSINE
type: UnificationXref
id: SARCOSINE
db: BioCyc

Compound/PW C001120 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: (R)-lipoic acid
stoichiometric coefficient: 1.0

Compound/PW C001144 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: NADH
stoichiometric coefficient: 1.0

Hydroxypyruvic acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Hydroxypyruvic acid

Reference/ChEBI CHEBI 18361
type: UnificationXref
id: CHEBI:18361
db: ChEBI

NADH
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: NADH

Reference/ChEBI CHEBI 18367
type: UnificationXref
id: CHEBI:18367
db: ChEBI

Reference/ChemSpider 1032
type: UnificationXref
id: 1032
db: ChemSpider

Reference/ChemSpider 1031
type: UnificationXref
id: 1031
db: ChemSpider

Reference/ChemSpider 388320
type: UnificationXref
id: 388320
db: ChemSpider

Reference/UniProt P13196
type: UnificationXref
id: P13196
db: UniProt

Cystathionine gamma-lyase
type: Protein
cellular location: Cytosol
entity reference: Cystathionine gamma-lyase

Compound/PW C000035 Mitochondrial Outer Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Ammonia
stoichiometric coefficient: 1.0

ChemicalStructure/PW C000905
type: ChemicalStructure
structure format: SMILES
structure data: CC(=O)C=O

Pyruvic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: Pyruvic acid
name: SubPathwayInput

NAD
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 15846
xref: Reference/HMDB HMDB0000902
xref: Reference/CAS 53-84-9
xref: Reference/KEGG Compound C00003
xref: Reference/PubChem-compound 5893
xref: Reference/BioCyc NAD
xref: Reference/ChemSpider 5682
chemical formula: C21H27N7O14P2
molecular weight: 663.10913
structure: ChemicalStructure/PW C000721

Reference/ChEBI CHEBI 17482
type: UnificationXref
id: CHEBI:17482
db: ChEBI

Adenosine triphosphate
type: SmallMolecule
cellular location: Cytoplasm
entity reference: Adenosine triphosphate

Reference/HMDB HMDB0000902
type: UnificationXref
id: HMDB0000902
db: HMDB

Succinyl-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Succinyl-CoA

Reference/HMDB HMDB0006454
type: UnificationXref
id: HMDB0006454
db: HMDB

RNA/26 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Seryl-tRNA(Ser)
stoichiometric coefficient: 1.0

Reference/ChemSpider 388301
type: UnificationXref
id: 388301
db: ChemSpider

Reference/ChemSpider 1057
type: UnificationXref
id: 1057
db: ChemSpider

Reference/ChemSpider 388307
type: UnificationXref
id: 388307
db: ChemSpider

Reference/KEGG Compound C00143
type: UnificationXref
id: C00143
db: KEGG Compound

Reference/CAS 56-88-2
type: UnificationXref
id: 56-88-2
db: CAS

Reference/ChemSpider 5653
type: UnificationXref
id: 5653
db: ChemSpider

Pyruvaldehyde
type: SmallMolecule
cellular location: Mitochondrial Outer Membrane
entity reference: Pyruvaldehyde

Reference/PubChem-compound 57689797
type: UnificationXref
id: 57689797
db: PubChem-compound

Guanidoacetic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: Guanidoacetic acid

L-Methionine
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000696
xref: Reference/ChEBI CHEBI 16643
xref: Reference/KEGG Compound C00073
xref: Reference/CAS 63-68-3
xref: Reference/BioCyc MET
xref: Reference/PubChem-compound 6137
xref: Reference/ChemSpider 5907
chemical formula: C5H11NO2S
molecular weight: 149.05106
structure: ChemicalStructure/PW C000548

Protein/447 Mitochondrion/Stoichiometry/2.0
type: Stoichiometry
physical entity: Glycine amidinotransferase, mitochondrial
stoichiometric coefficient: 2.0

D-Serine
type: SmallMoleculeReference
xref: Reference/ChemSpider 64231
xref: Reference/ChEBI CHEBI 16523
xref: Reference/BioCyc Serines
xref: Reference/HMDB HMDB0003406
xref: Reference/PubChem-compound 6857549
xref: Reference/CAS 312-84-5
xref: Reference/KEGG Compound C00740
chemical formula: C3H7NO3
molecular weight: 105.042595
structure: ChemicalStructure/PW C001875

Reference/KEGG Compound C01005
type: UnificationXref
id: C01005
db: KEGG Compound

Reference/PubChem-compound 880
type: UnificationXref
id: 880
db: PubChem-compound

ChemicalStructure/PW C001144
type: ChemicalStructure
structure format: SMILES

Reference/CAS 328-50-7
type: UnificationXref
id: 328-50-7
db: CAS

Reference/BioCyc AMP
type: UnificationXref
id: AMP
db: BioCyc

Reference/BioCyc GUANIDOACETIC ACID
type: UnificationXref
id: GUANIDOACETIC_ACID
db: BioCyc

Compound/PW C001099 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Coenzyme A
stoichiometric coefficient: 1.0

Reference/BioCyc HYDROGEN-PEROXIDE
type: UnificationXref
id: HYDROGEN-PEROXIDE
db: BioCyc

Glycine amidinotransferase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Glycine amidinotransferase, mitochondrial

Reference/HMDB HMDB0000250
type: UnificationXref
id: HMDB0000250
db: HMDB

Reference/HMDB HMDB0001341
type: UnificationXref
id: HMDB0001341
db: HMDB

Reference/UniProt P49591
type: UnificationXref
id: P49591
db: UniProt

Reference/BioCyc LIPOIC-ACID
type: UnificationXref
id: LIPOIC-ACID
db: BioCyc

Phosphoserine phosphatase
type: Protein
cellular location: Cytosol
entity reference: Phosphoserine phosphatase

Reference/KEGG Compound C00168
type: UnificationXref
id: C00168
db: KEGG Compound

Reference/ChemSpider 763
type: UnificationXref
id: 763
db: ChemSpider

ChemicalStructure/PW C000067
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CCSC[C@H](N)C(O)=O)C(O)=O

(R)-lipoic acid
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: (R)-lipoic acid

ChemicalStructure/PW C000062
type: ChemicalStructure
structure format: SMILES
structure data: CN(C)CC(O)=O

Compound/PW C000105 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Alanine
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0001352
type: UnificationXref
id: HMDB0001352
db: HMDB

Compound/PW C000120 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Serine
stoichiometric coefficient: 1.0

Reference/PubChem-compound 421
type: UnificationXref
id: 421
db: PubChem-compound

Reference/CAS 78-98-8
type: UnificationXref
id: 78-98-8
db: CAS

Ammonia
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Ammonia

Reference/UniProt P32929
type: UnificationXref
id: P32929
db: UniProt

Reference/CAS 7732-18-5
type: UnificationXref
id: 7732-18-5
db: CAS

Reference/KEGG Compound C01026
type: UnificationXref
id: C01026
db: KEGG Compound

Glycine amidinotransferase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P50440
organism: Homo sapiens

NAD
type: SmallMolecule
cellular location: Mitochondrion
entity reference: NAD

Reference/CAS 61-19-8
type: UnificationXref
id: 61-19-8
db: CAS

ChemicalStructure/PW C000032
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C001120
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CCCC[C@@H]1CCSS1

ChemicalStructure/PW C000030
type: ChemicalStructure
structure format: SMILES
structure data: C[N+](C)(C)CC([O-])=O

Glycyl-tRNA(Gly)
type: Rna
cellular location: Cytoplasm
entity reference: Glycyl-tRNA(Gly)

Reference/BioCyc AMMONIA
type: UnificationXref
id: AMMONIA
db: BioCyc

ChemicalStructure/PW C000035
type: ChemicalStructure
structure format: SMILES
structure data: N

Reference/CAS 56-86-0
type: UnificationXref
id: 56-86-0
db: CAS

Reference/CAS 63-68-3
type: UnificationXref
id: 63-68-3
db: CAS

Reference/HMDB HMDB0000271
type: UnificationXref
id: HMDB0000271
db: HMDB

Reference/HMDB HMDB0000272
type: UnificationXref
id: HMDB0000272
db: HMDB

NAD
type: SmallMolecule
cellular location: Cytosol
entity reference: NAD

Reference/CAS 56-40-6
type: UnificationXref
id: 56-40-6
db: CAS

RNA/29 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Glycyl-tRNA(Gly)
stoichiometric coefficient: 1.0

Sarcosine
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Sarcosine

Reference/HMDB HMDB0001377
type: UnificationXref
id: HMDB0001377
db: HMDB

S-Adenosylhomocysteine
type: SmallMolecule
cellular location: Cytosol
entity reference: S-Adenosylhomocysteine

Reference/HMDB HMDB0000045
type: UnificationXref
id: HMDB0000045
db: HMDB

Protein/612 Cytoplasm/Stoichiometry/2.0
type: Stoichiometry
physical entity: Serine--tRNA ligase, cytoplasmic
stoichiometric coefficient: 2.0

Compound/PW C000035 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Ammonia
stoichiometric coefficient: 1.0

Compound/PW C002747 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-2-Amino-3-oxobutanoic acid
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 17050
type: UnificationXref
id: CHEBI:17050
db: ChEBI

ChemicalStructure/PW C000046
type: ChemicalStructure
structure format: SMILES
structure data: CN(CC(O)=O)C(N)=N

Reference/HMDB HMDB0000043
type: UnificationXref
id: HMDB0000043
db: HMDB

Protein/635 Cytosol/Stoichiometry/2.0
type: Stoichiometry
physical entity: L-serine dehydratase/L-threonine deaminase
stoichiometric coefficient: 2.0

L-Alanine
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00041
xref: Reference/ChemSpider 5735
xref: Reference/ChEBI CHEBI 16977
xref: Reference/BioCyc L-ALPHA-ALANINE
xref: Reference/HMDB HMDB0000161
xref: Reference/CAS 56-41-7
xref: Reference/PubChem-compound 5950
chemical formula: C3H7NO2
molecular weight: 89.047676
structure: ChemicalStructure/PW C000105

Reference/ChemSpider 5800
type: UnificationXref
id: 5800
db: ChemSpider

Aminoacetone
type: SmallMoleculeReference
xref: Reference/PubChem-compound 215
xref: Reference/KEGG Compound C01888
xref: Reference/BioCyc AMINO-ACETONE
xref: Reference/ChemSpider 210
xref: Reference/ChEBI CHEBI 17906
xref: Reference/HMDB HMDB0002134
xref: Reference/CAS 298-08-8
chemical formula: C3H7NO
molecular weight: 73.052765
structure: ChemicalStructure/PW C001435

D-3-phosphoglycerate dehydrogenase
type: ProteinReference
xref: Reference/UniProt O43175
organism: Homo sapiens

Reference/ChemSpider 730
type: UnificationXref
id: 730
db: ChemSpider

Water
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Water

Reference/HMDB HMDB0001149
type: UnificationXref
id: HMDB0001149
db: HMDB

Aldehyde dehydrogenase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Aldehyde dehydrogenase, mitochondrial

ChemicalStructure/PW C000090
type: ChemicalStructure
structure format: SMILES
structure data: OC[C@@H](O)C(O)=O

Reference/HMDB HMDB0000051
type: UnificationXref
id: HMDB0000051
db: HMDB

Phosphoserine aminotransferase
type: Protein
cellular location: Cytosol
entity reference: Phosphoserine aminotransferase

ChemicalStructure/PW C000095
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CCC(O)=O)C(O)=O

βine
type: SmallMolecule
cellular location: Cytosol
entity reference: βine

Homocysteine
type: SmallMoleculeReference
xref: Reference/KEGG Compound C05330
xref: Reference/CAS 454-29-5
xref: Reference/HMDB HMDB0000742
xref: Reference/ChEBI CHEBI 17230
xref: Reference/ChemSpider 757
xref: Reference/PubChem-compound 49791978
xref: Reference/BioCyc HOMO-CYS
chemical formula: C4H9NO2S
molecular weight: 135.0354
structure: ChemicalStructure/PW C000590

Reference/KEGG Compound C02147
type: UnificationXref
id: C02147
db: KEGG Compound

Reference/CAS 58-64-0
type: UnificationXref
id: 58-64-0
db: CAS

Reference/KEGG Compound C03232
type: UnificationXref
id: C03232
db: KEGG Compound

Compound/PW C000590 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Homocysteine
stoichiometric coefficient: 1.0

L-serine dehydratase/L-threonine deaminase
type: ProteinReference
xref: Reference/UniProt P20132
organism: Homo sapiens

Reference/UniProt Q9GZT4
type: UnificationXref
id: Q9GZT4
db: UniProt

Reference/BioCyc HOMO-CYS
type: UnificationXref
id: HOMO-CYS
db: BioCyc

Compound/PW C000810 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Phosphohydroxypyruvic acid
stoichiometric coefficient: 1.0

L-Serine
type: SmallMoleculeReference
xref: Reference/ChemSpider 5736
xref: Reference/ChEBI CHEBI 17115
xref: Reference/CAS 56-45-1
xref: Reference/KEGG Compound C00065
xref: Reference/PubChem-compound 5951
xref: Reference/HMDB HMDB0000187
xref: Reference/BioCyc SER
chemical formula: C3H7NO3
molecular weight: 105.042595
structure: ChemicalStructure/PW C000120

Reference/HMDB HMDB0000064
type: UnificationXref
id: HMDB0000064
db: HMDB

Aldehyde dehydrogenase, mitochondrial
type: Complex
component stoichiometry: Protein/289 Mitochondrial Matrix/Stoichiometry/4.0
cellular location: Mitochondrial Matrix
component: Aldehyde dehydrogenase, mitochondrial

Reference/PubChem-compound 444493
type: UnificationXref
id: 444493
db: PubChem-compound

Reference/CAS 57-00-1
type: UnificationXref
id: 57-00-1
db: CAS

Reference/PubChem-compound 6083
type: UnificationXref
id: 6083
db: PubChem-compound

L-2-Amino-3-oxobutanoic acid
type: SmallMoleculeReference
xref: Reference/KEGG Compound C03508
xref: Reference/ChEBI CHEBI 40673
xref: Reference/PubChem-compound 440033
xref: Reference/HMDB HMDB0006454
xref: Reference/BioCyc AMINO-OXOBUT
xref: Reference/ChemSpider 389046
chemical formula: C4H7NO3
molecular weight: 117.042595
structure: ChemicalStructure/PW C002747

Reference/BioCyc 2-PG
type: UnificationXref
id: 2-PG
db: BioCyc

Reference/ChemSpider 757
type: UnificationXref
id: 757
db: ChemSpider

Reference/HMDB HMDB0001167
type: UnificationXref
id: HMDB0001167
db: HMDB

Reference/PubChem-compound 25246222
type: UnificationXref
id: 25246222
db: PubChem-compound

Glycine
type: SmallMolecule
cellular location: Cytosol
entity reference: Glycine

ChemicalStructure/PW C000075
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)C=O

Reference/BioCyc ACETYL-COA
type: UnificationXref
id: ACETYL-COA
db: BioCyc

Water
type: SmallMoleculeReference
xref: Reference/PubChem-compound 962
xref: Reference/ChEBI CHEBI 15377
xref: Reference/KEGG Compound C00001
xref: Reference/ChemSpider 937
xref: Reference/HMDB HMDB0002111
xref: Reference/CAS 7732-18-5
chemical formula: H2O
molecular weight: 18.010565
structure: ChemicalStructure/PW C001420

Dihydrolipoyl dehydrogenase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Dihydrolipoyl dehydrogenase, mitochondrial

ChemicalStructure/PW C000078
type: ChemicalStructure
structure format: SMILES
structure data: NCC(O)=O

Reference/BioCyc NAD
type: UnificationXref
id: NAD
db: BioCyc

Guanidinoacetate N-methyltransferase
type: ProteinReference
xref: Reference/UniProt Q14353
organism: Homo sapiens

Reference/ChemSpider 743
type: UnificationXref
id: 743
db: ChemSpider

Reference/BioCyc 5-AMINO-LEVULINATE
type: UnificationXref
id: 5-AMINO-LEVULINATE
db: BioCyc

Reference/BioCyc 3-P-SERINE
type: UnificationXref
id: 3-P-SERINE
db: BioCyc

Aminomethyltransferase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P48728
organism: Homo sapiens

Reference/ChemSpider 740
type: UnificationXref
id: 740
db: ChemSpider

ChemicalStructure/PW C001178
type: ChemicalStructure
structure format: SMILES

Aminoacetone
type: SmallMolecule
cellular location: Mitochondrial Outer Membrane
entity reference: Aminoacetone

ChemicalStructure/PW C000083
type: ChemicalStructure
structure format: SMILES
structure data: NC(=N)NCC(O)=O

Reference/PubChem-compound 247
type: UnificationXref
id: 247
db: PubChem-compound

Reference/CAS 2149-82-8
type: UnificationXref
id: 2149-82-8
db: CAS

Reference/ChEBI CHEBI 15842
type: UnificationXref
id: CHEBI:15842
db: ChEBI

Compound/PW C001178 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: 5,10-Methylene-THF
stoichiometric coefficient: 1.0

5-Aminolevulinic acid
type: SmallMolecule
cellular location: Mitochondrion
entity reference: 5-Aminolevulinic acid

ChemicalStructure/PW C002747
type: ChemicalStructure
structure format: SMILES
structure data: CC(=O)[C@H](N)C(O)=O

Sphingolipid Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway629
pathway component: SubPathwayInteraction629
xref: Reference/PathWhiz PW000148
xref: Reference/SMPDB SMP0000034
name: SubPathway

Compound/PW C000749 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: S-Adenosylhomocysteine
stoichiometric coefficient: 1.0

Compound/PW C000075 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Glyoxylic acid
stoichiometric coefficient: 1.0

Reference/ChemSpider 134
type: UnificationXref
id: 134
db: ChemSpider

ChemicalStructure/PW C001420
type: ChemicalStructure
structure format: SMILES
structure data: O

L-Arginine
type: SmallMoleculeReference
xref: Reference/BioCyc ARG
xref: Reference/ChEBI CHEBI 16467
xref: Reference/CAS 74-79-3
xref: Reference/KEGG Compound C00062
xref: Reference/ChemSpider 6082
xref: Reference/PubChem-compound 28782
xref: Reference/HMDB HMDB0000517
chemical formula: C6H14N4O2
molecular weight: 174.11168
structure: ChemicalStructure/PW C000396

D-Serine
type: SmallMolecule
cellular location: Cytosol
entity reference: D-Serine

Reference/CAS 473-81-4
type: UnificationXref
id: 473-81-4
db: CAS

Reference/ChEBI CHEBI 15611
type: UnificationXref
id: CHEBI:15611
db: ChEBI

Reference/CAS 62-46-4
type: UnificationXref
id: 62-46-4
db: CAS

Reference/ChemSpider 64231
type: UnificationXref
id: 64231
db: ChemSpider

Glycolysis
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway624
pathway component: SubPathwayInteraction624
xref: Reference/SMPDB SMP0000040
xref: Reference/PathWhiz PW000146
name: SubPathway

2-Ketobutyric acid
type: SmallMolecule
cellular location: Cytosol
entity reference: 2-Ketobutyric acid

Reference/ChEBI CHEBI 15846
type: UnificationXref
id: CHEBI:15846
db: ChEBI

Reference/BioCyc ADP
type: UnificationXref
id: ADP
db: BioCyc

Pyruvic acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 1060
xref: Reference/HMDB HMDB0000243
xref: Reference/CAS 127-17-3
xref: Reference/BioCyc PYRUVATE
xref: Reference/KEGG Compound C00022
xref: Reference/ChEBI CHEBI 32816
xref: Reference/ChemSpider 1031
chemical formula: C3H4O3
molecular weight: 88.016045
structure: ChemicalStructure/PW C000164

Glycine
type: SmallMolecule
cellular location: Cytoplasm
entity reference: Glycine

Glycine N-methyltransferase
type: Protein
cellular location: Cytosol
entity reference: Glycine N-methyltransferase

ChemicalStructure/PW C001875
type: ChemicalStructure
structure format: SMILES
structure data: N[C@H](CO)C(O)=O

ChemicalStructure/PW C000548
type: ChemicalStructure
structure format: SMILES
structure data: CSCC[C@H](N)C(O)=O

Reference/GENE ONTOLOGY GO 0005829
type: UnificationXref
id: GO:0005829
db: GENE ONTOLOGY

Tetrahydrofolic acid
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 20506
xref: Reference/ChemSpider 18714427
xref: Reference/HMDB HMDB0001846
xref: Reference/PubChem-compound 13781857
xref: Reference/BioCyc THF
xref: Reference/CAS 135-16-0
xref: Reference/KEGG Compound C00101
chemical formula: C19H23N7O6
molecular weight: 445.171
structure: ChemicalStructure/PW C001221

Oxoglutaric acid
type: SmallMoleculeReference
xref: Reference/CAS 328-50-7
xref: Reference/PubChem-compound 51
xref: Reference/BioCyc 2-KETOGLUTARATE
xref: Reference/HMDB HMDB0000208
xref: Reference/ChEBI CHEBI 30915
xref: Reference/KEGG Compound C00026
xref: Reference/ChemSpider 50
chemical formula: C5H6O5
molecular weight: 146.02153
structure: ChemicalStructure/PW C000134

Reference/ChEBI CHEBI 16908
type: UnificationXref
id: CHEBI:16908
db: ChEBI

Ammonia
type: SmallMolecule
cellular location: Cytosol
entity reference: Ammonia

Reference/BioCyc ADENOSYL-HOMO-CYS
type: UnificationXref
id: ADENOSYL-HOMO-CYS
db: BioCyc

Reaction669
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000548 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000030 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000590 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000062 Cytosol/Stoichiometry/1.0
right: Dimethylglycine
right: L-Methionine
spontaneous: false
left: βine
left: Homocysteine
name: Homocysteine + βine → Dimethylglycine + L-Methionine
conversion direction: LEFT_TO_RIGHT

Serine hydroxymethyltransferase, cytosolic
type: ProteinReference
xref: Reference/UniProt P34896
organism: Homo sapiens

Reference/ChEBI CHEBI 32398
type: UnificationXref
id: CHEBI:32398
db: ChEBI

Sarcosine dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q9UL12
organism: Homo sapiens

Serine hydroxymethyltransferase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P34897
organism: Homo sapiens

Reference/PubChem-compound 5862
type: UnificationXref
id: 5862
db: PubChem-compound

Serine--pyruvate aminotransferase
type: ProteinReference
xref: Reference/UniProt P21549
organism: Homo sapiens

Compound/PW C000644 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: 3-Phosphoglyceric acid
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0001533
type: UnificationXref
id: HMDB0001533
db: HMDB

Compound/PW C000062 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Dimethylglycine
stoichiometric coefficient: 1.0

Reference/ChemSpider 62074
type: UnificationXref
id: 62074
db: ChemSpider

Reference/CAS 600-18-0
type: UnificationXref
id: 600-18-0
db: CAS

Pyruvate Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway621
pathway component: SubPathwayInteraction621
xref: Reference/SMPDB SMP0000060
xref: Reference/PathWhiz PW000054
name: SubPathway

Orotidylic acid
type: SmallMoleculeReference
xref: Reference/CAS 2149-82-8
xref: Reference/BioCyc OROTIDINE-5-PHOSPHATE
xref: Reference/PubChem-compound 160617
xref: Reference/ChemSpider 141140
xref: Reference/ChEBI CHEBI 15842
xref: Reference/HMDB HMDB0000218
xref: Reference/KEGG Compound C01103
chemical formula: C10H13N2O11P
molecular weight: 368.0257
structure: ChemicalStructure/PW C000144

Mitochondrion
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005739

Reference/BioCyc THF
type: UnificationXref
id: THF
db: BioCyc

Phosphohydroxypyruvic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: Phosphohydroxypyruvic acid

Reference/ChEBI CHEBI 16919
type: UnificationXref
id: CHEBI:16919
db: ChEBI

Reference/BioCyc GLY
type: UnificationXref
id: GLY
db: BioCyc

Reference/ChEBI CHEBI 16977
type: UnificationXref
id: CHEBI:16977
db: ChEBI

Guanidoacetic acid
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Guanidoacetic acid

Reference/HMDB HMDB0000208
type: UnificationXref
id: HMDB0000208
db: HMDB

ReactionCatalysis1518
type: Catalysis
controller: 5-aminolevulinate synthase, nonspecific, mitochondrial
controlled: 2.3.1.37
control type: ACTIVATION

Glyceric acid
type: SmallMolecule
cellular location: Cytosol
entity reference: Glyceric acid

ChemicalStructure/PW C001221
type: ChemicalStructure
structure format: SMILES

ReactionCatalysis1522
type: Catalysis
controller: D-3-phosphoglycerate dehydrogenase
controlled: 1.1.1.95
control type: ACTIVATION

ReactionCatalysis1521
type: Catalysis
controller: Glycerate kinase
controlled: 2.7.1.31
control type: ACTIVATION

ReactionCatalysis1524
type: Catalysis
controller: Phosphoserine phosphatase
controlled: 3.1.3.3
control type: ACTIVATION

Hydroxypyruvic acid
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 30841
xref: Reference/CAS 1113-60-6
xref: Reference/BioCyc OH-PYR
xref: Reference/PubChem-compound 964
xref: Reference/ChemSpider 939
xref: Reference/KEGG Compound C00168
xref: Reference/HMDB HMDB0001352
chemical formula: C3H4O4
molecular weight: 104.010956
structure: ChemicalStructure/PW C001043

ReactionCatalysis1523
type: Catalysis
controller: Phosphoserine aminotransferase
controlled: 2.6.1.52
control type: ACTIVATION

Reference/HMDB HMDB0000696
type: UnificationXref
id: HMDB0000696
db: HMDB

ReactionCatalysis1526
type: Catalysis
controller: Serine--tRNA ligase, cytoplasmic
controlled: 6.1.1.11
control type: ACTIVATION

ReactionCatalysis1525
type: Catalysis
controller: L-serine dehydratase/L-threonine deaminase
controlled: Reaction1789
control type: ACTIVATION

ChemicalStructure/PW C000134
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CCC(=O)C(O)=O

ReactionCatalysis1528
type: Catalysis
controller: Glycine--tRNA ligase
controlled: 6.1.1.14
control type: ACTIVATION

ReactionCatalysis1527
type: Catalysis
controller: Serine hydroxymethyltransferase, cytosolic
controlled: 2.1.2.1
control type: ACTIVATION

Reference/CAS 124-38-9
type: UnificationXref
id: 124-38-9
db: CAS

L-Alanine
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: L-Alanine

ReactionCatalysis1520
type: Catalysis
controller: Glycine N-methyltransferase
controlled: 2.1.1.20
control type: ACTIVATION

Reference/ChEBI CHEBI 15414
type: UnificationXref
id: CHEBI:15414
db: ChEBI

Reference/PubChem-compound 6857549
type: UnificationXref
id: 6857549
db: PubChem-compound

Reference/HMDB HMDB0000218
type: UnificationXref
id: HMDB0000218
db: HMDB

ReactionCatalysis1529
type: Catalysis
controller: Serine racemase
controlled: Reaction1793
control type: ACTIVATION

Compound/PW C000721 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: NAD
stoichiometric coefficient: 1.0

Reference/PubChem-compound 673
type: UnificationXref
id: 673
db: PubChem-compound

Reference/ChemSpider 566
type: UnificationXref
id: 566
db: ChemSpider

Compound/PW C000186 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Phosphoserine
stoichiometric coefficient: 1.0

Reference/PubChem-compound 5257127
type: UnificationXref
id: 5257127
db: PubChem-compound

Reference/CAS 1113-60-6
type: UnificationXref
id: 1113-60-6
db: CAS

ChemicalStructure/PW C000144
type: ChemicalStructure
structure format: SMILES

Reference/CAS 7664-41-7
type: UnificationXref
id: 7664-41-7
db: CAS

Compound/PW C000046 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Creatine
stoichiometric coefficient: 1.0

Reference/BioCyc GLT
type: UnificationXref
id: GLT
db: BioCyc

L-serine dehydratase/L-threonine deaminase
type: Complex
component stoichiometry: Protein/635 Cytosol/Stoichiometry/2.0
cellular location: Cytosol
component: L-serine dehydratase/L-threonine deaminase

Serine hydroxymethyltransferase, mitochondrial
type: Protein
cellular location: Mitochondrial Matrix
entity reference: Serine hydroxymethyltransferase, mitochondrial

RNA/28 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Glycyl-tRNA
stoichiometric coefficient: 1.0

Serine racemase
type: Protein
cellular location: Cytosol
entity reference: Serine racemase

Reference/CAS 462-20-4
type: UnificationXref
id: 462-20-4
db: CAS

1.8.1.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001144 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001120 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C007757 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrion/Stoichiometry/1.0
right: (R)-lipoic acid
right: NADH
spontaneous: false
EC number: 1.8.1.4
left: NAD
left: Dihydrolipoate
name: Dihydrolipoate + NAD → (R)-lipoic acid + NADH
conversion direction: LEFT_TO_RIGHT

Reference/BioCyc S-ADENOSYLMETHIONINE
type: UnificationXref
id: S-ADENOSYLMETHIONINE
db: BioCyc

ChemicalStructure/PW C000105
type: ChemicalStructure
structure format: SMILES
structure data: C[C@H](N)C(O)=O

ChemicalStructure/PW C001435
type: ChemicalStructure
structure format: SMILES
structure data: CC(=O)CN

Reference/PubChem-compound 222
type: UnificationXref
id: 222
db: PubChem-compound

Reference/HMDB HMDB0012210
type: UnificationXref
id: HMDB0012210
db: HMDB

Compound/PW C001420 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Water
stoichiometric coefficient: 1.0

Pyrophosphate
type: SmallMoleculeReference
xref: Reference/PubChem-compound 644102
xref: Reference/BioCyc PPI
xref: Reference/ChemSpider 559142
xref: Reference/ChEBI CHEBI 18361
xref: Reference/HMDB HMDB0000250
xref: Reference/CAS 14000-31-8
xref: Reference/KEGG Compound C00013
chemical formula: H4O7P2
molecular weight: 177.94322
structure: ChemicalStructure/PW C000170

Reference/BioCyc OH-PYR
type: UnificationXref
id: OH-PYR
db: BioCyc

Reference/ChemSpider 6082
type: UnificationXref
id: 6082
db: ChemSpider

Creatine
type: SmallMolecule
cellular location: Cytosol
entity reference: Creatine

ChemicalStructure/PW C000590
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CCS)C(O)=O

Glycine--tRNA ligase
type: Complex
component stoichiometry: Protein/536 Cytoplasm/Stoichiometry/2.0
cellular location: Cytoplasm
component: Glycine--tRNA ligase

Cysteine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway609
pathway component: SubPathwayInteraction609
xref: Reference/SMPDB SMP0000013
xref: Reference/PathWhiz PW000018
name: SubPathway

Reference/PubChem-compound 215
type: UnificationXref
id: 215
db: PubChem-compound

Reference/ChEBI CHEBI 15636
type: UnificationXref
id: CHEBI:15636
db: ChEBI

Reference/HMDB HMDB0000243
type: UnificationXref
id: HMDB0000243
db: HMDB

ChemicalStructure/PW C000120
type: ChemicalStructure
structure format: SMILES
structure data: N[C@@H](CO)C(O)=O

Compound/PW C001144 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: NADH
stoichiometric coefficient: 1.0

Reference/ChemSpider 103
type: UnificationXref
id: 103
db: ChemSpider

Adenosine triphosphate
type: SmallMolecule
cellular location: Cytosol
entity reference: Adenosine triphosphate

Reference/UniProt P48728
type: UnificationXref
id: P48728
db: UniProt

Compound/PW C000078 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Glycine
stoichiometric coefficient: 1.0

Adenosine diphosphate
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00008
xref: Reference/PubChem-compound 6022
xref: Reference/ChemSpider 5800
xref: Reference/CAS 58-64-0
xref: Reference/BioCyc ADP
xref: Reference/ChEBI CHEBI 16761
xref: Reference/HMDB HMDB0001341
chemical formula: C10H15N5O10P2
molecular weight: 427.02942
structure: ChemicalStructure/PW C001034

Compound/PW C000940 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Acetyl-CoA
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0000005
type: UnificationXref
id: HMDB0000005
db: HMDB

Compound/PW C001783 Mitochondrial Outer Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Hydrogen peroxide
stoichiometric coefficient: 1.0

Phosphoserine
type: SmallMoleculeReference
xref: Reference/PubChem-compound 57689797
xref: Reference/BioCyc 3-P-SERINE
xref: Reference/ChemSpider 62074
xref: Reference/ChEBI CHEBI 15811
xref: Reference/CAS 407-41-0
xref: Reference/HMDB HMDB0000272
xref: Reference/KEGG Compound C01005
chemical formula: C3H8NO6P
molecular weight: 185.00893
structure: ChemicalStructure/PW C000186

Compound/PW C001435 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Aminoacetone
stoichiometric coefficient: 1.0

Sarcosine
type: SmallMoleculeReference
xref: Reference/CAS 107-97-1
xref: Reference/BioCyc SARCOSINE
xref: Reference/ChemSpider 1057
xref: Reference/ChEBI CHEBI 15611
xref: Reference/HMDB HMDB0000271
xref: Reference/KEGG Compound C00213
xref: Reference/PubChem-compound 1088
chemical formula: C3H7NO2
molecular weight: 89.047676
structure: ChemicalStructure/PW C000185

Mitochondrial Outer Membrane
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005741

Reference/SMPDB SMP0000128
type: UnificationXref
id: SMP0000128
db: SMPDB

ReactionCatalysis1160
type: Catalysis
controller: Aminomethyltransferase, mitochondrial
controlled: 2.1.2.10
control type: ACTIVATION

Compound/PW C000905 Mitochondrial Outer Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyruvaldehyde
stoichiometric coefficient: 1.0

Compound/PW C000083 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Guanidoacetic acid
stoichiometric coefficient: 1.0

Protein/739 Cytosol/Stoichiometry/4.0
type: Stoichiometry
physical entity: Serine hydroxymethyltransferase, cytosolic
stoichiometric coefficient: 4.0

ChemicalStructure/PW C000721
type: ChemicalStructure
structure format: SMILES

Reference/BioCyc CPD-8587
type: UnificationXref
id: CPD-8587
db: BioCyc

Reference/HMDB HMDB0000807
type: UnificationXref
id: HMDB0000807
db: HMDB

Reference/ChEBI CHEBI 30831
type: UnificationXref
id: CHEBI:30831
db: ChEBI

Reaction1770
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000075 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000078 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000164 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000105 Mitochondrial Matrix/Stoichiometry/1.0
right: Glyoxylic acid
right: L-Alanine
spontaneous: false
left: Glycine
left: Pyruvic acid
name: Glycine + Pyruvic acid ↔ Glyoxylic acid + L-Alanine
conversion direction: REVERSIBLE

Reference/ChEBI CHEBI 1146900
type: UnificationXref
id: CHEBI:1146900
db: ChEBI

2.3.1.37
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000808 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000078 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000894 Mitochondrion/Stoichiometry/1.0
right: Coenzyme A
right: 5-Aminolevulinic acid
spontaneous: false
EC number: 2.3.1.37
left: Succinyl-CoA
left: Glycine
name: Glycine + Succinyl-CoA → 5-Aminolevulinic acid + Coenzyme A
conversion direction: LEFT_TO_RIGHT

2.1.1.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000749 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000083 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000921 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000046 Cytosol/Stoichiometry/1.0
right: Creatine
right: S-Adenosylmethionine
spontaneous: false
EC number: 2.1.1.2
left: S-Adenosylhomocysteine
left: Guanidoacetic acid
name: Guanidoacetic acid + S-Adenosylhomocysteine → Creatine + S-Adenosylmethionine
conversion direction: LEFT_TO_RIGHT

2.1.4.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000396 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000078 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000083 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000144 Mitochondrion/Stoichiometry/1.0
right: Orotidylic acid
right: Guanidoacetic acid
spontaneous: false
EC number: 2.1.4.1
left: Glycine
left: L-Arginine
name: Glycine + L-Arginine ↔ Guanidoacetic acid + Orotidylic acid
conversion direction: REVERSIBLE

Reference/UniProt P78330
type: UnificationXref
id: P78330
db: UniProt

Water
type: SmallMolecule
cellular location: Mitochondrial Outer Membrane
entity reference: Water

Reaction1769
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001043 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000164 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000120 Mitochondrial Matrix/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000105 Mitochondrial Matrix/Stoichiometry/1.0
right: Hydroxypyruvic acid
right: L-Alanine
spontaneous: false
left: Pyruvic acid
left: L-Serine
name: L-Serine + Pyruvic acid ↔ Hydroxypyruvic acid + L-Alanine
conversion direction: REVERSIBLE

Reference/PubChem-compound 1088
type: UnificationXref
id: 1088
db: PubChem-compound

S-Adenosylmethionine
type: SmallMolecule
cellular location: Cytosol
entity reference: S-Adenosylmethionine

Coenzyme A
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 1146900
xref: Reference/HMDB HMDB0001423
xref: Reference/PubChem-compound 6816
xref: Reference/BioCyc CO-A
xref: Reference/ChemSpider 6557
xref: Reference/CAS 85-61-0
xref: Reference/KEGG Compound C00010
chemical formula: C21H36N7O16P3S
molecular weight: 767.11523
structure: ChemicalStructure/PW C001099

Adenosine diphosphate
type: SmallMolecule
cellular location: Cytosol
entity reference: Adenosine diphosphate

Cytoplasm
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005737

Serine--tRNA ligase, cytoplasmic
type: Protein
cellular location: Cytoplasm
entity reference: Serine--tRNA ligase, cytoplasmic

ReactionCatalysis1174
type: Catalysis
controller: Serine hydroxymethyltransferase, mitochondrial
controlled: 2.1.2.1
control type: ACTIVATION

Glycine
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Glycine

Reference/ChEBI CHEBI 15422
type: UnificationXref
id: CHEBI:15422
db: ChEBI

Coenzyme A
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Coenzyme A

Compound/PW C000721 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: NAD
stoichiometric coefficient: 1.0

Homo sapiens
type: BioSource
xref: Reference/TAXONOMY 9606

ChemicalStructure/PW C000940
type: ChemicalStructure
structure format: SMILES

Reference/ChemSpider 388299
type: UnificationXref
id: 388299
db: ChemSpider

Compound/PW C001034 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Adenosine diphosphate
stoichiometric coefficient: 1.0

6.1.1.14
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000170 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000078 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: RNA/28 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: RNA/29 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000414 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000032 Cytoplasm/Stoichiometry/1.0
right: Adenosine monophosphate
right: Glycyl-tRNA(Gly)
right: Pyrophosphate
spontaneous: false
EC number: 6.1.1.14
left: Glycine
left: Glycyl-tRNA
left: Adenosine triphosphate
name: Adenosine triphosphate + Glycine + Glycyl-tRNA → Adenosine monophosphate + Glycyl-tRNA(Gly) + Pyrophosphate
conversion direction: LEFT_TO_RIGHT

Reaction1793
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000120 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001875 Cytosol/Stoichiometry/1.0
right: D-Serine
spontaneous: false
left: L-Serine
name: L-Serine → D-Serine
conversion direction: LEFT_TO_RIGHT

Oxygen
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001377
xref: Reference/ChemSpider 952
xref: Reference/KEGG Compound C00007
xref: Reference/ChEBI CHEBI 15379
xref: Reference/PubChem-compound 977
xref: Reference/BioCyc CPD-6641
xref: Reference/CAS 7782-44-7
chemical formula: O2
molecular weight: 31.98983
structure: ChemicalStructure/PW C001065

6.1.1.11
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000120 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000170 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: RNA/26 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: RNA/27 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000414 Cytoplasm/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000032 Cytoplasm/Stoichiometry/1.0
right: Adenosine monophosphate
right: L-Seryl-tRNA(Ser)
right: Pyrophosphate
spontaneous: false
EC number: 6.1.1.11
left: Adenosine triphosphate
left: L-Serine
left: L-Seryl-tRNA
name: Adenosine triphosphate + L-Serine + L-Seryl-tRNA → Adenosine monophosphate + L-Seryl-tRNA(Ser) + Pyrophosphate
conversion direction: LEFT_TO_RIGHT

2.1.2.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000120 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001221 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001178 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000078 Cytosol/Stoichiometry/1.0
right: Glycine
right: 5,10-Methylene-THF
right: Water
spontaneous: false
EC number: 2.1.2.1
left: Tetrahydrofolic acid
left: L-Serine
name: L-Serine + Tetrahydrofolic acid ↔ 5,10-Methylene-THF + Glycine + Water
conversion direction: REVERSIBLE

Reference/ChEBI CHEBI 40673
type: UnificationXref
id: CHEBI:40673
db: ChEBI

Reference/ChEBI CHEBI 16523
type: UnificationXref
id: CHEBI:16523
db: ChEBI

Reference/ChEBI CHEBI 16526
type: UnificationXref
id: CHEBI:16526
db: ChEBI

Compound/PW C000095 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Glutamic acid
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 16761
type: UnificationXref
id: CHEBI:16761
db: ChEBI

2.1.1.20
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000749 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000185 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000921 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000078 Cytosol/Stoichiometry/1.0
right: Glycine
right: S-Adenosylmethionine
spontaneous: false
EC number: 2.1.1.20
left: S-Adenosylhomocysteine
left: Sarcosine
name: S-Adenosylhomocysteine + Sarcosine ↔ Glycine + S-Adenosylmethionine
conversion direction: REVERSIBLE

Reference/UniProt P20132
type: UnificationXref
id: P20132
db: UniProt

Reaction1789
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000120 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000035 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000164 Cytosol/Stoichiometry/1.0
right: Ammonia
right: Pyruvic acid
spontaneous: false
left: L-Serine
name: L-Serine ↔ Ammonia + Pyruvic acid
conversion direction: REVERSIBLE

Reference/ChEBI CHEBI 15428
type: UnificationXref
id: CHEBI:15428
db: ChEBI

2.6.1.52
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000186 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000134 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000095 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000810 Cytosol/Stoichiometry/1.0
right: Oxoglutaric acid
right: Phosphoserine
spontaneous: false
EC number: 2.6.1.52
left: Phosphohydroxypyruvic acid
left: L-Glutamic acid
name: L-Glutamic acid + Phosphohydroxypyruvic acid ↔ Oxoglutaric acid + Phosphoserine
conversion direction: REVERSIBLE

3.1.3.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000120 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000186 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001104 Cytosol/Stoichiometry/1.0
right: Phosphate
right: L-Serine
spontaneous: false
EC number: 3.1.3.3
left: Phosphoserine
left: Water
name: Phosphoserine + Water → L-Serine + Phosphate
conversion direction: LEFT_TO_RIGHT

Compound/PW C001420 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Water
stoichiometric coefficient: 1.0

Serine--tRNA ligase, cytoplasmic
type: Complex
component stoichiometry: Protein/612 Cytoplasm/Stoichiometry/2.0
cellular location: Cytoplasm
component: Serine--tRNA ligase, cytoplasmic

1.1.1.95
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000644 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000810 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Cytosol/Stoichiometry/1.0
right: NADH
right: Phosphohydroxypyruvic acid
spontaneous: false
EC number: 1.1.1.95
left: NAD
left: 3-Phosphoglyceric acid
name: 3-Phosphoglyceric acid + NAD ↔ NADH + Phosphohydroxypyruvic acid
conversion direction: REVERSIBLE

Non-Ketotic Hyperglycinemia
type: Pathway
pathway order: PathwayEnzymeReaction/2088
pathway order: PathwayEnzymeReaction/2122
pathway order: PathwayEnzymeReaction/2110600
pathway order: PathwayEnzymeReaction/2140
pathway order: PathwayEnzymeReaction/2118
pathway order: PathwayEnzymeReaction/1587
pathway order: PathwaySubPathway/609
pathway order: PathwayEnzymeReaction/2138
pathway order: PathwaySubPathway/629
pathway order: PathwayEnzymeReaction/2117
pathway order: PathwayEnzymeReaction/2125747
pathway order: PathwayEnzymeReaction/2133
pathway order: PathwayEnzymeReaction/2134
pathway order: PathwayEnzymeReaction/2135
pathway order: PathwayEnzymeReaction/2110611
pathway order: PathwaySubPathway/623
pathway order: PathwaySubPathway/624
pathway order: PathwayEnzymeReaction/2130
pathway order: PathwaySubPathway/621
pathway order: PathwayEnzymeReaction/1534
pathway order: PathwayEnzymeReaction/2129
pathway order: PathwayEnzymeReaction/2511930
pathway order: PathwayEnzymeReaction/2511932
pathway order: PathwayEnzymeReaction/1531
pathway order: PathwayEnzymeReaction/2511931
pathway order: PathwayEnzymeReaction/2127
pathway order: PathwayEnzymeReaction/1535
pathway order: PathwayEnzymeReaction/1519
pathway order: PathwayEnzymeReaction/1539
pathway order: PathwayEnzymeReaction/2511934
pathway order: PathwayEnzymeReaction/211577
pathway order: PathwayEnzymeReaction/2511933
pathway order: PathwayEnzymeReaction/211578
pathway component: ReactionCatalysis1160
pathway component: ReactionCatalysis1518
pathway component: ReactionCatalysis1522
pathway component: Reaction1770
pathway component: Sphingolipid Metabolism
pathway component: ReactionCatalysis1521
pathway component: ReactionCatalysis1524
pathway component: ReactionCatalysis1523
pathway component: ReactionCatalysis1526
pathway component: ReactionCatalysis1525
pathway component: ReactionCatalysis1528
pathway component: ReactionCatalysis1527
pathway component: 1.4.3.4
pathway component: 2.3.1.37
pathway component: 2.1.1.2
pathway component: 2.1.2.1
pathway component: ReactionCatalysis1520
pathway component: 2.1.4.1
pathway component: Reaction1769
pathway component: ReactionCatalysis1529
pathway component: Glycolysis
pathway component: Gluconeogenesis
pathway component: ReactionCatalysis1174
pathway component: ReactionCatalysis1135
pathway component: ReactionCatalysis1498
pathway component: 2.1.2.10
pathway component: 4.4.1.1
pathway component: Reaction815
pathway component: 1.8.1.4
pathway component: 6.1.1.14
pathway component: 2.3.1.29
pathway component: Reaction1793
pathway component: 6.1.1.11
pathway component: ReactionCatalysis1149
pathway component: 1.4.4.2
pathway component: 1.5.8.3
pathway component: ReactionCatalysis1146
pathway component: 1.5.8.4
pathway component: Cysteine Metabolism
pathway component: Reaction669
pathway component: ReactionCatalysis363
pathway component: Reaction1429
pathway component: 2.1.1.20
pathway component: ReactionCatalysis1513
pathway component: ReactionCatalysis1515

2.7.1.31
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000090 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000644 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000414 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001034 Cytosol/Stoichiometry/1.0
right: Adenosine diphosphate
right: 3-Phosphoglyceric acid
spontaneous: false
EC number: 2.7.1.31
left: Adenosine triphosphate
left: Glyceric acid
name: Adenosine triphosphate + Glyceric acid ↔ 3-Phosphoglyceric acid + Adenosine diphosphate
conversion direction: REVERSIBLE

Reference/PathWhiz PW000148
type: UnificationXref
id: PW000148
db: PathWhiz

Reference/PathWhiz PW000146
type: UnificationXref
id: PW000146
db: PathWhiz

Reference/PubChem-compound 5893
type: UnificationXref
id: 5893
db: PubChem-compound

Compound/PW C001178 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 5,10-Methylene-THF
stoichiometric coefficient: 1.0

Compound/PW C008526 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid
stoichiometric coefficient: 1.0

Dihydrolipoate
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Dihydrolipoate

Compound/PW C002747 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-2-Amino-3-oxobutanoic acid
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 32816
type: UnificationXref
id: CHEBI:32816
db: ChEBI

L-Serine
type: SmallMolecule
cellular location: Cytosol
entity reference: L-Serine
name: SubPathwayInput

Compound/PW C000032 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Adenosine monophosphate
stoichiometric coefficient: 1.0

Reference/ChEBI CHEBI 16344
type: UnificationXref
id: CHEBI:16344
db: ChEBI

Compound/PW C000414 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Adenosine triphosphate
stoichiometric coefficient: 1.0

Phosphate
type: SmallMolecule
cellular location: Cytosol
entity reference: Phosphate

Reference/PubChem-compound 280
type: UnificationXref
id: 280
db: PubChem-compound

8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid
type: SmallMoleculeReference
xref: Reference/PubChem-compound 53481912
xref: Reference/HMDB HMDB0013639
chemical formula: C9H19NO2S2
molecular weight: 237.08572
structure: ChemicalStructure/PW C008526

L-Serine
type: SmallMolecule
cellular location: Cytoplasm
entity reference: L-Serine

ReactionCatalysis1135
type: Catalysis
controller: Amine oxidase [flavin-containing] A
controlled: 1.4.3.4
control type: ACTIVATION

Reference/PubChem-compound 25243997
type: UnificationXref
id: 25243997
db: PubChem-compound

ChemicalStructure/PW C000749
type: ChemicalStructure
structure format: SMILES

ReactionCatalysis1149
type: Catalysis
controller: 2-amino-3-ketobutyrate coenzyme A ligase, mitochondrial
controlled: 2.3.1.29
control type: ACTIVATION

Reference/CAS 407-41-0
type: UnificationXref
id: 407-41-0
db: CAS

ReactionCatalysis1146
type: Catalysis
controller: Aldehyde dehydrogenase, mitochondrial
controlled: 1.2.1.3
control type: ACTIVATION

Reference/PubChem-compound 3946848
type: UnificationXref
id: 3946848
db: PubChem-compound

Reference/PubChem-compound 1060
type: UnificationXref
id: 1060
db: PubChem-compound

Reference/PubChem-compound 1061
type: UnificationXref
id: 1061
db: PubChem-compound

ReactionCatalysis363
type: Catalysis
controller: Cystathionine gamma-lyase
controlled: 4.4.1.1
control type: ACTIVATION

Reference/HMDB HMDB0001967
type: UnificationXref
id: HMDB0001967
db: HMDB

Reference/UniProt P41250
type: UnificationXref
id: P41250
db: UniProt

Reference/PubChem-compound 24762165
type: UnificationXref
id: 24762165
db: PubChem-compound

Reference/ChEBI CHEBI 30841
type: UnificationXref
id: CHEBI:30841
db: ChEBI

ReactionCatalysis1151
type: Catalysis
controller: Dimethylglycine dehydrogenase, mitochondrial
controlled: 1.5.8.4
control type: ACTIVATION

Reference/PubChem-compound 13781857
type: UnificationXref
id: 13781857
db: PubChem-compound

ReactionCatalysis1153
type: Catalysis
controller: Sarcosine dehydrogenase, mitochondrial
controlled: 1.5.8.3
control type: ACTIVATION

ReactionCatalysis1157
type: Catalysis
controller: Glycine dehydrogenase [decarboxylating], mitochondrial
controlled: 1.4.4.2
control type: ACTIVATION