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

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

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

S-Methylmalonyl-CoA
type: SmallMolecule
cellular location: Cytosol
entity reference: S-Methylmalonyl-CoA

Methionine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway109082
pathway component: SubPathwayInteraction109082
xref: Reference/PathWhiz PW000056
xref: Reference/SMPDB SMP0000033
name: SubPathway

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

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

Protein/16488 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Probable methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
stoichiometric coefficient: 1.0

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 C000988 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Propionyl-CoA
stoichiometric coefficient: 1.0

Reference/BioCyc D-METHYL-MALONYL-COA
type: UnificationXref
id: D-METHYL-MALONYL-COA
db: BioCyc

Reference/SMPDB SMP0087516
type: UnificationXref
id: SMP0087516
db: SMPDB

Reference/PubChem-compound 58838673
type: UnificationXref
id: 58838673
db: PubChem-compound

Reference/UniProt O44451
type: UnificationXref
id: O44451
db: UniProt

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

Reference/PathWhiz PW000056
type: UnificationXref
id: PW000056
db: PathWhiz

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

Reference/KEGG Compound C00683
type: UnificationXref
id: C00683
db: KEGG Compound

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

Uncharacterized protein
type: Protein
cellular location: Cytosol
entity reference: Uncharacterized protein

Probable methylmalonyl-CoA mutase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Probable methylmalonyl-CoA mutase, mitochondrial

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

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

ChemicalStructure/PW C000463
type: ChemicalStructure
structure format: SMILES
structure data: OC(O)=O

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

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

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

Reference/PubChem-compound 6288
type: UnificationXref
id: 6288
db: PubChem-compound

Reference/BioCyc HCO3
type: UnificationXref
id: HCO3
db: BioCyc

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

Protein/16469 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
stoichiometric coefficient: 1.0

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

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

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

L-serine dehydratase/L-threonine deaminase
type: Complex
component stoichiometry: Protein/15257 Cytosol/Stoichiometry/2.0
cellular location: Cytosol
component: Uncharacterized protein

Compound/PW C000109 Extracellular Space/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Threonine
stoichiometric coefficient: 1.0

Reference/BioCyc ALPHA-GLUCOSE-16-BISPHOSPHATE
type: UnificationXref
id: ALPHA-GLUCOSE-16-BISPHOSPHATE
db: BioCyc

Reference/UniProt P34385
type: UnificationXref
id: P34385
db: UniProt

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

Citric Acid Cycle
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway109083
pathway component: SubPathwayInteraction109083
xref: Reference/PathWhiz PW000005
xref: Reference/SMPDB SMP0000057
name: SubPathway

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

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

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

Reference/KEGG Compound C00100
type: UnificationXref
id: C00100
db: KEGG Compound

ChemicalStructure/PW C001523
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C001099
type: ChemicalStructure
structure format: SMILES

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

branched-chain alpha-keto dehydrogenase complex
type: Complex
component stoichiometry: Protein/16478 Mitochondrion/Stoichiometry/1.0
component stoichiometry: Protein/14858 Mitochondrion/Stoichiometry/1.0
component stoichiometry: Protein/16469 Mitochondrion/Stoichiometry/1.0
component stoichiometry: Protein/14850 Mitochondrion/Stoichiometry/1.0
cellular location: Mitochondrion
component: Dihydrolipoyl dehydrogenase, mitochondrial
component: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
component: Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
component: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

Reference/PubChem-compound 439164
type: UnificationXref
id: 439164
db: PubChem-compound

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

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

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

ChemicalStructure/PW C000808
type: ChemicalStructure
structure format: SMILES

Methylmalonyl-CoA Epimerase
type: ProteinReference
xref: Reference/UniProt G5EEG4
organism: Caenorhabditis elegans

Propionyl-CoA
type: SmallMoleculeReference
xref: Reference/CAS 317-66-8
xref: Reference/PubChem-compound 439164
xref: Reference/KEGG Compound C00100
xref: Reference/ChEBI CHEBI 15539
xref: Reference/BioCyc PROPIONYL-COA
xref: Reference/ChemSpider 388310
xref: Reference/HMDB HMDB0001275
chemical formula: C24H40N7O17P3S
molecular weight: 823.1414
structure: ChemicalStructure/PW C000988

Probable methylmalonyl-CoA mutase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q23381
organism: Caenorhabditis elegans

Reference/ChEBI CHEBI 16857
type: UnificationXref
id: CHEBI:16857
db: ChEBI

Reference/KEGG Compound C01213
type: UnificationXref
id: C01213
db: KEGG Compound

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

Reference/ChemSpider 388310
type: UnificationXref
id: 388310
db: ChemSpider

ReactionCatalysis139714
type: Catalysis
controller: Methylmalonyl-CoA Epimerase
controlled: 5.1.99.1
control type: ACTIVATION

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

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

Reference/CAS 72-19-5
type: UnificationXref
id: 72-19-5
db: CAS

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

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

Methylmalonyl-CoA Epimerase
type: Protein
cellular location: Cytosol
entity reference: Methylmalonyl-CoA Epimerase

Reference/CAS 73173-92-9
type: UnificationXref
id: 73173-92-9
db: CAS

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

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

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

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

ReactionCatalysis139728
type: Catalysis
controller: branched-chain alpha-keto dehydrogenase complex
controlled: 2.3.1.168
control type: ACTIVATION

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

ReactionCatalysis139727
type: Catalysis
controller: Propionyl-CoA carboxylase
controlled: 6.4.1.3
control type: ACTIVATION

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

Reference/ChemSpider 6051
type: UnificationXref
id: 6051
db: ChemSpider

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

Reference/SMPDB SMP0000057
type: UnificationXref
id: SMP0000057
db: SMPDB

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial

Hydrogen
type: SmallMoleculeReference
xref: Reference/PubChem-compound 58838673
xref: Reference/CAS 1333-74-0
xref: Reference/ChemSpider 762
xref: Reference/BioCyc ALPHA-GLUCOSE-16-BISPHOSPHATE
xref: Reference/KEGG Compound C00282
xref: Reference/HMDB HMDB0001362
xref: Reference/ChEBI CHEBI 18276
chemical formula: H2
molecular weight: 2.01565
structure: ChemicalStructure/PW C001051

Succinyl-CoA
type: SmallMolecule
cellular location: Cytosol
entity reference: Succinyl-CoA
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/CAS 73173-91-8
type: UnificationXref
id: 73173-91-8
db: CAS

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

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

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

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

ChemicalStructure/PW C000109
type: ChemicalStructure
structure format: SMILES
structure data: C[C@@H](O)[C@H](N)C(O)=O

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

Extracellular Space
type: CellularLocationVocabulary
xref: Reference/GENE ONTOLOGY GO 0005615

Reference/HMDB HMDB0000595
type: UnificationXref
id: HMDB0000595
db: HMDB

ChemicalStructure/PW C001560
type: ChemicalStructure
structure format: SMILES

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

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

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

Compound/PW C001523 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: R-Methylmalonyl-CoA
stoichiometric coefficient: 1.0

Compound/PW C000463 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Hydrogen carbonate
stoichiometric coefficient: 1.0

Reference/UniProt Q19842
type: UnificationXref
id: Q19842
db: UniProt

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

Reference/PubChem-compound 22833590
type: UnificationXref
id: 22833590
db: PubChem-compound

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

Reaction149527
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000003 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000035 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000109 Cytosol/Stoichiometry/1.0
right: Ammonia
right: 2-Ketobutyric acid
spontaneous: false
left: L-Threonine
name: L-Threonine → 2-Ketobutyric acid + Ammonia
conversion direction: LEFT_TO_RIGHT

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

5.4.99.2
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000808 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001523 Mitochondrion/Stoichiometry/1.0
right: Succinyl-CoA
spontaneous: false
EC number: 5.4.99.2
left: R-Methylmalonyl-CoA
name: R-Methylmalonyl-CoA → Succinyl-CoA
conversion direction: LEFT_TO_RIGHT

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

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

Reference/CAS 317-66-8
type: UnificationXref
id: 317-66-8
db: CAS

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

L-Threonine
type: SmallMolecule
cellular location: Extracellular Space
entity reference: L-Threonine

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

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

Reference/GENE ONTOLOGY GO 0005615
type: UnificationXref
id: GO:0005615
db: GENE ONTOLOGY

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

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

Reference/UniProt O45924
type: UnificationXref
id: O45924
db: UniProt

Protein/14850 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
stoichiometric coefficient: 1.0

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

Reference/PubChem-compound 769
type: UnificationXref
id: 769
db: PubChem-compound

S-Methylmalonyl-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: S-Methylmalonyl-CoA

ChemicalStructure/PW C000721
type: ChemicalStructure
structure format: SMILES

R-Methylmalonyl-CoA
type: SmallMolecule
cellular location: Mitochondrion
entity reference: R-Methylmalonyl-CoA

Reference/ChEBI CHEBI 18276
type: UnificationXref
id: CHEBI:18276
db: ChEBI

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

Reference/PathWhiz PW088535
type: UnificationXref
id: PW088535
db: PathWhiz

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

ChemicalStructure/PW C001144
type: ChemicalStructure
structure format: SMILES

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

Protein/16478 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
stoichiometric coefficient: 1.0

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

Reference/KEGG Compound C00282
type: UnificationXref
id: C00282
db: KEGG Compound

Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial

Reference/ChemSpider 17216177
type: UnificationXref
id: 17216177
db: ChemSpider

Protein/15257 Cytosol/Stoichiometry/2.0
type: Stoichiometry
physical entity: Uncharacterized protein
stoichiometric coefficient: 2.0

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

Reference/HMDB HMDB0002310
type: UnificationXref
id: HMDB0002310
db: HMDB

Reference/PubChem-compound 21252287
type: UnificationXref
id: 21252287
db: PubChem-compound

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

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

R-Methylmalonyl-CoA
type: SmallMoleculeReference
xref: Reference/BioCyc METHYL-MALONYL-COA
xref: Reference/PubChem-compound 22833590
xref: Reference/ChemSpider 17216177
xref: Reference/CAS 73173-92-9
xref: Reference/KEGG Compound C01213
xref: Reference/HMDB HMDB0002255
chemical formula: C25H40N7O19P3S
molecular weight: 867.1312
structure: ChemicalStructure/PW C001523

ReactionCatalysis139924
type: Catalysis
controller: Methylmalonyl-CoA mutase, mitochondrial
controlled: 5.4.99.2
control type: ACTIVATION

ReactionCatalysis139923
type: Catalysis
controller: L-serine dehydratase/L-threonine deaminase
controlled: Reaction149527
control type: ACTIVATION

Reference/KEGG Compound C00288
type: UnificationXref
id: C00288
db: KEGG Compound

ChemicalStructure/PW C001034
type: ChemicalStructure
structure format: SMILES

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

Reference/ChemSpider 762
type: UnificationXref
id: 762
db: ChemSpider

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

Caenorhabditis elegans
type: BioSource
xref: Reference/TAXONOMY 6239

Threonine and 2-Oxobutanoate Degradation
type: Pathway
pathway order: PathwayEnzymeReaction/2117205
pathway order: PathwayEnzymeReaction/2117206
pathway order: PathwayEnzymeReaction/2117207
pathway order: PathwayTransport/2117209
pathway order: PathwayEnzymeReaction/2117203
pathway order: PathwayEnzymeReaction/2117204
pathway order: PathwaySubPathway/109082
pathway order: PathwaySubPathway/109081
pathway order: PathwaySubPathway/109083
pathway order: PathwayTransport/2117208
pathway component: 6.4.1.3
pathway component: 2.3.1.168
pathway component: Reaction149527
pathway component: 5.1.99.1
pathway component: 5.4.99.2
pathway component: ReactionCatalysis139924
pathway component: ReactionCatalysis139923
pathway component: ReactionCatalysis139714
pathway component: ReactionCatalysis139728
pathway component: ReactionCatalysis139727
pathway component: Methionine Metabolism
pathway component: Transport8276
pathway component: Transport8277
pathway component: Homocysteine Degradation
pathway component: Citric Acid Cycle
xref: Reference/PathWhiz PW088535
xref: Reference/SMPDB SMP0087516
organism: Caenorhabditis elegans

Probable methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Probable methylcrotonoyl-CoA carboxylase beta chain, mitochondrial

Reference/TAXONOMY 6239
type: UnificationXref
id: 6239
db: TAXONOMY

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

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

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

Hydrogen carbonate
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Hydrogen carbonate

5.1.99.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001560 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001523 Cytosol/Stoichiometry/1.0
right: R-Methylmalonyl-CoA
spontaneous: false
EC number: 5.1.99.1
left: S-Methylmalonyl-CoA
name: S-Methylmalonyl-CoA → R-Methylmalonyl-CoA
conversion direction: LEFT_TO_RIGHT

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

Reference/UniProt Q23571
type: UnificationXref
id: Q23571
db: UniProt

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

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

Transport8276
type: Transport
participant stoichiometry: Compound/PW C000109 Extracellular Space/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000109 Cytosol/Stoichiometry/1.0
right: L-Threonine
spontaneous: false
left: L-Threonine
name: L-Threonine (→) Transport: Caenorhabditis elegans, Extracellular Space to Caenorhabditis elegans, Cell, Cytosol
conversion direction: LEFT_TO_RIGHT

Transport8277
type: Transport
participant stoichiometry: Compound/PW C000003 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000003 Mitochondrion/Stoichiometry/1.0
right: 2-Ketobutyric acid
spontaneous: false
left: 2-Ketobutyric acid
name: 2-Ketobutyric acid (→) Transport: Caenorhabditis elegans, Cell, Cytosol to Caenorhabditis elegans, Cell, Mitochondrion
conversion direction: LEFT_TO_RIGHT

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

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

Reference/ChEBI CHEBI 15539
type: UnificationXref
id: CHEBI:15539
db: ChEBI

Reference/HMDB HMDB0001362
type: UnificationXref
id: HMDB0001362
db: HMDB

Reference/UniProt D3YT81
type: UnificationXref
id: D3YT81
db: UniProt

Homocysteine Degradation
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway109081
pathway component: SubPathwayInteraction109081
xref: Reference/PathWhiz PW000027
xref: Reference/SMPDB SMP0000455
name: SubPathway

Propionyl-CoA carboxylase
type: Complex
component stoichiometry: Protein/16488 Mitochondrion/Stoichiometry/1.0
component stoichiometry: Protein/16364 Mitochondrion/Stoichiometry/1.0
cellular location: Mitochondrion
component: Probable methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
component: Propionyl-CoA carboxylase alpha chain, mitochondrial

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

Protein/16509 Mitochondrion/Stoichiometry/2.0
type: Stoichiometry
physical entity: Probable methylmalonyl-CoA mutase, mitochondrial
stoichiometric coefficient: 2.0

Reference/UniProt Q95XY8
type: UnificationXref
id: Q95XY8
db: UniProt

Reference/KEGG Compound C00188
type: UnificationXref
id: C00188
db: KEGG Compound

Compound/PW C001560 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: S-Methylmalonyl-CoA
stoichiometric coefficient: 1.0

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

Reference/HMDB HMDB0000167
type: UnificationXref
id: HMDB0000167
db: HMDB

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

Reference/UniProt G5EEG4
type: UnificationXref
id: G5EEG4
db: UniProt

Propionyl-CoA carboxylase alpha chain, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Propionyl-CoA carboxylase alpha chain, mitochondrial

Compound/PW C001523 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: R-Methylmalonyl-CoA
stoichiometric coefficient: 1.0

Methylmalonyl-CoA mutase, mitochondrial
type: Complex
component stoichiometry: Protein/16509 Mitochondrion/Stoichiometry/2.0
cellular location: Mitochondrion
component: Probable methylmalonyl-CoA mutase, mitochondrial

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

Reference/SMPDB SMP0000033
type: UnificationXref
id: SMP0000033
db: SMPDB

L-Threonine
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000167
xref: Reference/PubChem-compound 6288
xref: Reference/CAS 72-19-5
xref: Reference/ChemSpider 6051
xref: Reference/ChEBI CHEBI 16857
xref: Reference/KEGG Compound C00188
xref: Reference/BioCyc THR
chemical formula: C4H9NO3
molecular weight: 119.05824
structure: ChemicalStructure/PW C000109

Reference/CAS 1333-74-0
type: UnificationXref
id: 1333-74-0
db: CAS

6.4.1.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000463 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001051 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000988 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001034 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001560 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000414 Mitochondrion/Stoichiometry/1.0
right: S-Methylmalonyl-CoA
right: Adenosine diphosphate
right: Hydrogen
spontaneous: false
EC number: 6.4.1.3
left: Adenosine triphosphate
left: Propionyl-CoA
left: Hydrogen carbonate
name: Adenosine triphosphate + Hydrogen carbonate + Propionyl-CoA → Adenosine diphosphate + Hydrogen + S-Methylmalonyl-CoA
conversion direction: LEFT_TO_RIGHT

2.3.1.168
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001144 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001099 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000988 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000003 Mitochondrion/Stoichiometry/1.0
right: Propionyl-CoA
right: NADH
spontaneous: false
EC number: 2.3.1.168
left: NAD
left: 2-Ketobutyric acid
left: Coenzyme A
name: 2-Ketobutyric acid + Coenzyme A + NAD → NADH + Propionyl-CoA
conversion direction: LEFT_TO_RIGHT

Reference/ChEBI CHEBI 17544
type: UnificationXref
id: CHEBI:17544
db: ChEBI

Uncharacterized protein
type: ProteinReference
xref: Reference/UniProt Q95XY8
organism: Caenorhabditis elegans

Reference/PathWhiz PW000027
type: UnificationXref
id: PW000027
db: PathWhiz

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

Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial

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

S-Methylmalonyl-CoA
type: SmallMoleculeReference
xref: Reference/CAS 73173-91-8
xref: Reference/KEGG Compound C00683
xref: Reference/BioCyc D-METHYL-MALONYL-COA
xref: Reference/ChemSpider 13628334
xref: Reference/HMDB HMDB0002310
xref: Reference/PubChem-compound 21252287
chemical formula: C25H40N7O19P3S
molecular weight: 867.1312
structure: ChemicalStructure/PW C001560

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

Reference/ChemSpider 13628334
type: UnificationXref
id: 13628334
db: ChemSpider

2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
type: ProteinReference
xref: Reference/UniProt O45924
organism: Caenorhabditis elegans

Protein/16364 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Propionyl-CoA carboxylase alpha chain, mitochondrial
stoichiometric coefficient: 1.0

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

Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
type: ProteinReference
xref: Reference/UniProt O44451
organism: Caenorhabditis elegans

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

ChemicalStructure/PW C000414
type: ChemicalStructure
structure format: SMILES

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

Reference/BioCyc METHYL-MALONYL-COA
type: UnificationXref
id: METHYL-MALONYL-COA
db: BioCyc

SubPathwayInteraction109081
type: Interaction
name: SubPathwayReaction
name: SubPathway109081Reaction
participant: 2-Ketobutyric acid

SubPathwayInteraction109083
type: Interaction
name: SubPathway109083Reaction
name: SubPathwayReaction
participant: Succinyl-CoA

Protein/14858 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Dihydrolipoyl dehydrogenase, mitochondrial
stoichiometric coefficient: 1.0

SubPathwayInteraction109082
type: Interaction
name: SubPathwayReaction
name: SubPathway109082Reaction
participant: 2-Ketobutyric acid

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/SMPDB SMP0000455
type: UnificationXref
id: SMP0000455
db: SMPDB

Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q23571
organism: Caenorhabditis elegans

Dihydrolipoyl dehydrogenase, mitochondrial
type: ProteinReference
xref: Reference/UniProt D3YT81
organism: Caenorhabditis elegans

Reference/HMDB HMDB0001275
type: UnificationXref
id: HMDB0001275
db: HMDB

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

Reference/PathWhiz PW000005
type: UnificationXref
id: PW000005
db: PathWhiz

ChemicalStructure/PW C000988
type: ChemicalStructure
structure format: SMILES

R-Methylmalonyl-CoA
type: SmallMolecule
cellular location: Cytosol
entity reference: R-Methylmalonyl-CoA

Compound/PW C001051 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Hydrogen
stoichiometric coefficient: 1.0

Propionyl-CoA carboxylase alpha chain, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q19842
organism: Caenorhabditis elegans

Probable methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
type: ProteinReference
xref: Reference/UniProt P34385
organism: Caenorhabditis elegans

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

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

Reference/HMDB HMDB0002255
type: UnificationXref
id: HMDB0002255
db: HMDB

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

Reference/ChemSpider 749
type: UnificationXref
id: 749
db: ChemSpider

Reference/CAS 71-52-3
type: UnificationXref
id: 71-52-3
db: CAS

Compound/PW C001560 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: S-Methylmalonyl-CoA
stoichiometric coefficient: 1.0

Reference/UniProt Q23381
type: UnificationXref
id: Q23381
db: UniProt

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

Hydrogen carbonate
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0000595
xref: Reference/ChEBI CHEBI 17544
xref: Reference/PubChem-compound 769
xref: Reference/BioCyc HCO3
xref: Reference/ChemSpider 749
xref: Reference/CAS 71-52-3
xref: Reference/KEGG Compound C00288
chemical formula: CH2O3
molecular weight: 62.000393
structure: ChemicalStructure/PW C000463

ChemicalStructure/PW C001051
type: ChemicalStructure
structure format: SMILES
structure data: [H][H]

Compound/PW C000109 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: L-Threonine
stoichiometric coefficient: 1.0