Reference/UniProt P09601
type: UnificationXref
id: P09601
db: UniProt

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

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

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

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

Porphobilinogen deaminase
type: Protein
cellular location: Cytosol
entity reference: Porphobilinogen deaminase

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

Reference/BioCyc UROPORPHYRIN III
type: UnificationXref
id: UROPORPHYRIN_III
db: BioCyc

Reference/BioCyc COPROPORPHYRINOGEN I
type: UnificationXref
id: COPROPORPHYRINOGEN_I
db: BioCyc

Reference/PubChem-compound 29936
type: UnificationXref
id: 29936
db: PubChem-compound

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

Reference/HMDB HMDB0002158
type: UnificationXref
id: HMDB0002158
db: HMDB

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

ChemicalStructure/PW C002078
type: ChemicalStructure
structure format: SMILES

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

Reference/KEGG Compound C00448
type: UnificationXref
id: C00448
db: KEGG Compound

Reference/PathWhiz PW000175
type: UnificationXref
id: PW000175
db: PathWhiz

Uroporphyrinogen III
type: SmallMolecule
cellular location: Cytosol
entity reference: Uroporphyrinogen III

Reference/CAS 607-14-7
type: UnificationXref
id: 607-14-7
db: CAS

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

Compound/PW C000863 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Protoporphyrinogen IX
stoichiometric coefficient: 1.0

Reference/ChemSpider 1146
type: UnificationXref
id: 1146
db: ChemSpider

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

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/ChEBI CHEBI 17200
type: UnificationXref
id: CHEBI:17200
db: ChEBI

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

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

Reference/HMDB HMDB0001086
type: UnificationXref
id: HMDB0001086
db: HMDB

Reference/BioCyc UDP-GLUCURONATE
type: UnificationXref
id: UDP-GLUCURONATE
db: BioCyc

Reference/PubChem-compound 6031
type: UnificationXref
id: 6031
db: PubChem-compound

Transport1220
type: Transport
participant stoichiometry: Compound/PW C000163 Mitochondrial Inner Membrane/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000163 Mitochondrial Matrix/Stoichiometry/1.0
right: Protoporphyrin IX
spontaneous: false
left: Protoporphyrin IX
name: Protoporphyrin IX (←) Transport: Homo sapiens, Cell, Mitochondrial Inner Membrane to Homo sapiens, Cell, Mitochondrial Matrix
conversion direction: LEFT_TO_RIGHT

Reference/PathWhiz PW000157
type: UnificationXref
id: PW000157
db: PathWhiz

Compound/PW C000797 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Biliverdin
stoichiometric coefficient: 1.0

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

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

Reference/PubChem-compound 445713
type: UnificationXref
id: 445713
db: PubChem-compound

Reference/UniProt P10746
type: UnificationXref
id: P10746
db: UniProt

Compound/PW C000746 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Uridine diphosphate glucuronic acid
stoichiometric coefficient: 1.0

Reference/ChemSpider 27815
type: UnificationXref
id: 27815
db: ChemSpider

Reference/UniProt P22830
type: UnificationXref
id: P22830
db: UniProt

Fe2+
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Fe2+

Uridine 5'-diphosphate
type: SmallMolecule
cellular location: Cytosol
entity reference: Uridine 5'-diphosphate

Reference/CAS 531-14-6
type: UnificationXref
id: 531-14-6
db: CAS

Reference/ChEBI CHEBI 27484
type: UnificationXref
id: CHEBI:27484
db: ChEBI

Reference/HMDB HMDB0001097
type: UnificationXref
id: HMDB0001097
db: HMDB

ChemicalStructure/PW C000808
type: ChemicalStructure
structure format: SMILES

Bilirubin diglucuronide
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0003325
xref: Reference/PubChem-compound 5459911
xref: Reference/ChemSpider 4573640
xref: Reference/ChEBI CHEBI 18392
xref: Reference/CAS 17459-92-6
xref: Reference/BioCyc BILIRUBIN-BISGLUCURONOSIDE
xref: Reference/KEGG Compound C05787
chemical formula: C45H52N4O18
molecular weight: 936.32764
structure: ChemicalStructure/PW C001838

Reference/ChemSpider 389645
type: UnificationXref
id: 389645
db: ChemSpider

Uroporphyrin I
type: SmallMoleculeReference
xref: Reference/ChemSpider 16736725
xref: Reference/CAS 607-14-7
xref: Reference/BioCyc UROPORPHYRIN I
xref: Reference/KEGG Compound C05767
xref: Reference/HMDB HMDB0000936
xref: Reference/ChEBI CHEBI 27484
chemical formula: C40H38N4O16
molecular weight: 830.2283
structure: ChemicalStructure/PW C000747

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

Reference/ChemSpider 389644
type: UnificationXref
id: 389644
db: ChemSpider

Uridine diphosphate glucuronic acid
type: SmallMoleculeReference
xref: Reference/BioCyc UDP-GLUCURONATE
xref: Reference/CAS 2616-64-0
xref: Reference/PubChem-compound 17473
xref: Reference/HMDB HMDB0000935
xref: Reference/ChEBI CHEBI 17200
xref: Reference/ChemSpider 16522
xref: Reference/KEGG Compound C00167
chemical formula: C15H22N2O18P2
molecular weight: 580.0343
structure: ChemicalStructure/PW C000746

Reference/KEGG Compound C00237
type: UnificationXref
id: C00237
db: KEGG Compound

Coproporphyrin I
type: SmallMoleculeReference
xref: Reference/ChemSpider 16736701
xref: Reference/HMDB HMDB0000643
xref: Reference/CAS 531-14-6
xref: Reference/KEGG Compound C05769
xref: Reference/ChEBI CHEBI 28421
chemical formula: C36H38N4O8
molecular weight: 654.269
structure: ChemicalStructure/PW C000502

Reference/ChemSpider 389649
type: UnificationXref
id: 389649
db: ChemSpider

Protoporphyrinogen IX
type: SmallMoleculeReference
xref: Reference/CAS 7412-77-3
xref: Reference/ChemSpider 108741
xref: Reference/BioCyc PROTOPORPHYRINOGEN
xref: Reference/ChEBI CHEBI 15435
xref: Reference/KEGG Compound C01079
xref: Reference/PubChem-compound 121893
xref: Reference/HMDB HMDB0001097
chemical formula: C34H40N4O4
molecular weight: 568.30493
structure: ChemicalStructure/PW C000863

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

Reference/ChEBI CHEBI 17381
type: UnificationXref
id: CHEBI:17381
db: ChEBI

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

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

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

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

Compound/PW C003003 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Heme A
stoichiometric coefficient: 1.0

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

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

Reference/ChEBI CHEBI 17033
type: UnificationXref
id: CHEBI:17033
db: ChEBI

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

Reference/KEGG Compound C00486
type: UnificationXref
id: C00486
db: KEGG Compound

Protoporphyrin IX
type: SmallMolecule
cellular location: Mitochondrial Matrix
entity reference: Protoporphyrin IX

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

Reference/CAS 114-25-0
type: UnificationXref
id: 114-25-0
db: CAS

Uroporphyrinogen-III synthase
type: ProteinReference
xref: Reference/UniProt P10746
organism: Homo sapiens

UDP-glucuronosyltransferase 2B11
type: ProteinReference
xref: Reference/UniProt O75310
organism: Homo sapiens

Porphobilinogen deaminase
type: ProteinReference
xref: Reference/UniProt P08397
organism: Homo sapiens

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

Protoporphyrinogen IX
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Protoporphyrinogen IX

Reference/HMDB HMDB0003178
type: UnificationXref
id: HMDB0003178
db: HMDB

Compound/PW C000201 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Uridine 5'-diphosphate
stoichiometric coefficient: 1.0

Transport178
type: Transport
participant stoichiometry: Compound/PW C000974 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000974 Cytosol/Stoichiometry/1.0
right: Coproporphyrinogen III
spontaneous: false
left: Coproporphyrinogen III
name: Coproporphyrinogen III (→) Transport: Homo sapiens, Cell, Cytosol to Homo sapiens, Cell, Mitochondrion
conversion direction: LEFT_TO_RIGHT

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

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

Reference/ChEBI CHEBI 17245
type: UnificationXref
id: CHEBI:17245
db: ChEBI

Reference/KEGG Compound C00015
type: UnificationXref
id: C00015
db: KEGG Compound

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

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

ChemicalStructure/PW C000900
type: ChemicalStructure
structure format: SMILES

Uroporphyrinogen decarboxylase
type: ProteinReference
xref: Reference/UniProt P06132
organism: Homo sapiens

Ferritin, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Ferritin, mitochondrial

Coproporphyrinogen-III oxidase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P36551
organism: Homo sapiens

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

Feline leukemia virus subgroup C receptor-related protein 2
type: ProteinReference
xref: Reference/UniProt Q9UPI3

Reference/HMDB HMDB0004158
type: UnificationXref
id: HMDB0004158
db: HMDB

Compound/PW C000900 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Heme O
stoichiometric coefficient: 1.0

Reference/UniProt Q12887
type: UnificationXref
id: Q12887
db: UniProt

Reference/ChEBI CHEBI 17499
type: UnificationXref
id: CHEBI:17499
db: ChEBI

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

Uroporphyrinogen III
type: SmallMoleculeReference
xref: Reference/ChemSpider 1146
xref: Reference/KEGG Compound C01051
xref: Reference/CAS 1976-85-8
xref: Reference/BioCyc UROPORPHYRINOGEN-III
xref: Reference/ChEBI CHEBI 15437
xref: Reference/PubChem-compound 1179
xref: Reference/HMDB HMDB0001086
chemical formula: C40H44N4O16
molecular weight: 836.2752
structure: ChemicalStructure/PW C000854

Reference/CAS 18273-06-8
type: UnificationXref
id: 18273-06-8
db: CAS

Reference/BioCyc CPD-10134
type: UnificationXref
id: CPD-10134
db: BioCyc

Uroporphyrin III
type: SmallMoleculeReference
xref: Reference/ChemSpider 16736727
xref: Reference/CAS 18273-06-8
xref: Reference/ChEBI CHEBI 15436
xref: Reference/KEGG Compound C02469
xref: Reference/HMDB HMDB0000916
xref: Reference/BioCyc UROPORPHYRIN III
chemical formula: C40H38N4O16
molecular weight: 830.2283
structure: ChemicalStructure/PW C000732

Coproporphyrinogen III
type: SmallMoleculeReference
xref: Reference/KEGG Compound C03263
xref: Reference/ChEBI CHEBI 15439
xref: Reference/HMDB HMDB0001261
xref: Reference/BioCyc COPROPORPHYRINOGEN III
xref: Reference/PubChem-compound 321
xref: Reference/CAS 2624-63-7
xref: Reference/ChemSpider 315
chemical formula: C36H44N4O8
molecular weight: 660.3159
structure: ChemicalStructure/PW C000974

Protoporphyrinogen IX
type: SmallMolecule
entity reference: Protoporphyrinogen IX

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

Reference/UniProt O75310
type: UnificationXref
id: O75310
db: UniProt

Reference/HMDB HMDB0000916
type: UnificationXref
id: HMDB0000916
db: HMDB

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

Reference/CAS 553-12-8
type: UnificationXref
id: 553-12-8
db: CAS

Compound/PW C000732 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Uroporphyrin III
stoichiometric coefficient: 1.0

Compound/PW C000163/Stoichiometry/1.0
type: Stoichiometry
physical entity: Protoporphyrin IX
stoichiometric coefficient: 1.0

Uroporphyrinogen I
type: SmallMolecule
cellular location: Cytosol
entity reference: Uroporphyrinogen I

Compound/Generic66 Cytosol/Stoichiometry/3.0
type: Stoichiometry
physical entity: A
stoichiometric coefficient: 3.0

Fe2+
type: SmallMoleculeReference
xref: Reference/CAS 15438-31-0
xref: Reference/KEGG Compound C14818
xref: Reference/ChemSpider 25394
xref: Reference/PubChem-compound 27284
xref: Reference/ChEBI CHEBI 29033
xref: Reference/BioCyc Ferric-Hydroxamate-Complexes
xref: Reference/HMDB HMDB0000692
chemical formula: Fe
molecular weight: 55.93494
structure: ChemicalStructure/PW C000544

ReactionCatalysis1717
type: Catalysis
controller: Ferrochelatase, mitochondrial
controlled: 4.99.1.1
control type: ACTIVATION

Reference/CAS 73023-76-4
type: UnificationXref
id: 73023-76-4
db: CAS

Reference/CAS 372-97-4
type: UnificationXref
id: 372-97-4
db: CAS

ReactionCatalysis1716
type: Catalysis
controller: Protoporphyrinogen oxidase
controlled: 1.3.3.4
control type: ACTIVATION

Reference/KEGG Compound C00032
type: UnificationXref
id: C00032
db: KEGG Compound

ReactionCatalysis1718
type: Catalysis
controller: Ferritin, mitochondrial
controlled: 1.16.3.1
control type: ACTIVATION

Uroporphyrin III
type: SmallMolecule
cellular location: Cytosol
entity reference: Uroporphyrin III

UDP-glucuronosyltransferase 2B11
type: Protein
cellular location: Cytosol
entity reference: UDP-glucuronosyltransferase 2B11

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

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

Heme oxygenase 1
type: Protein
cellular location: Cytosol
entity reference: Heme oxygenase 1

ChemicalStructure/PW C001144
type: ChemicalStructure
structure format: SMILES

Biliverdin reductase A
type: ProteinReference
xref: Reference/UniProt P53004
organism: Homo sapiens

Hydrogen peroxide
type: SmallMolecule
entity reference: Hydrogen peroxide

Compound/PW C001051 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: Hydrogen
stoichiometric coefficient: 4.0

Beta-glucuronidase
type: ProteinReference
xref: Reference/UniProt P08236
organism: Homo sapiens

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

Compound/PW C000544 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: Fe2+
stoichiometric coefficient: 4.0

Reference/ChEBI CHEBI 18392
type: UnificationXref
id: CHEBI:18392
db: ChEBI

ReactionCatalysis1726
type: Catalysis
controller: Protoheme IX farnesyltransferase, mitochondrial
controlled: 2.5.1.-
control type: ACTIVATION

Reference/UniProt P50336
type: UnificationXref
id: P50336
db: UniProt

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

AH2
type: SmallMolecule
cellular location: Cytosol
entity reference: AH2

Biliverdin
type: SmallMoleculeReference
xref: Reference/PubChem-compound 5353439
xref: Reference/BioCyc BILIVERDINE
xref: Reference/CAS 114-25-0
xref: Reference/HMDB HMDB0001008
xref: Reference/KEGG Compound C00500
xref: Reference/ChEBI CHEBI 17033
xref: Reference/ChemSpider 4510089
chemical formula: C33H34N4O6
molecular weight: 582.24786
structure: ChemicalStructure/PW C000797

Compound/PW C000166 Cytosol/Stoichiometry/4.0
type: Stoichiometry
physical entity: Porphobilinogen
stoichiometric coefficient: 4.0

Reference/KEGG Compound C02469
type: UnificationXref
id: C02469
db: KEGG Compound

Reference/ChemSpider 767
type: UnificationXref
id: 767
db: ChemSpider

Reference/KEGG Compound C00167
type: UnificationXref
id: C00167
db: KEGG Compound

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

Compound/PW C000863/Stoichiometry/1.0
type: Stoichiometry
physical entity: Protoporphyrinogen IX
stoichiometric coefficient: 1.0

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

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

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

Uridine 5'-diphosphate
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 17659
xref: Reference/PubChem-compound 6031
xref: Reference/CAS 58-98-0
xref: Reference/HMDB HMDB0000295
xref: Reference/KEGG Compound C00015
xref: Reference/ChemSpider 5809
xref: Reference/BioCyc UDP
chemical formula: C9H14N2O12P2
molecular weight: 404.0022
structure: ChemicalStructure/PW C000201

Farnesyl pyrophosphate
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00448
xref: Reference/HMDB HMDB0000961
xref: Reference/ChemSpider 393270
xref: Reference/ChEBI CHEBI 17407
xref: Reference/CAS 372-97-4
xref: Reference/BioCyc FARNESYL-PP
xref: Reference/PubChem-compound 445713
chemical formula: C15H28O7P2
molecular weight: 382.13104
structure: ChemicalStructure/PW C000768

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

Hydroxymethylbilane
type: SmallMoleculeReference
xref: Reference/HMDB HMDB0001137
xref: Reference/ChEBI CHEBI 16645
xref: Reference/PubChem-compound 788
xref: Reference/KEGG Compound C01024
xref: Reference/CAS 73023-76-4
xref: Reference/BioCyc HYDROXYMETHYLBILANE
xref: Reference/ChemSpider 767
chemical formula: C40H46N4O17
molecular weight: 854.28577
structure: ChemicalStructure/PW C000887

Reference/KEGG Compound C15672
type: UnificationXref
id: C15672
db: KEGG Compound

Compound/PW C000163 Mitochondrial Inner Membrane/Stoichiometry/1.0
type: Stoichiometry
physical entity: Protoporphyrin IX
stoichiometric coefficient: 1.0

Reference/PubChem-compound 788
type: UnificationXref
id: 788
db: PubChem-compound

Reference/KEGG Compound C15670
type: UnificationXref
id: C15670
db: KEGG Compound

Reference/KEGG Compound C01024
type: UnificationXref
id: C01024
db: KEGG Compound

Uroporphyrinogen-III synthase
type: Protein
cellular location: Cytosol
entity reference: Uroporphyrinogen-III synthase

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

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

Reference/BioCyc BILIRUBIN-BISGLUCURONOSIDE
type: UnificationXref
id: BILIRUBIN-BISGLUCURONOSIDE
db: BioCyc

Coproporphyrin I
type: SmallMolecule
cellular location: Cytosol
entity reference: Coproporphyrin I

Reference/ChemSpider 315
type: UnificationXref
id: 315
db: ChemSpider

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

ReactionCatalysis1741
type: Catalysis
controller: Cytochrome c oxidase assembly protein COX15 homolog
controlled: Reaction1911
control type: ACTIVATION

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

Reference/CAS 17208-65-0
type: UnificationXref
id: 17208-65-0
db: CAS

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

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

Reference/HMDB HMDB0001008
type: UnificationXref
id: HMDB0001008
db: HMDB

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

Reference/ChEBI CHEBI 29033
type: UnificationXref
id: CHEBI:29033
db: ChEBI

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

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

Reference/HMDB HMDB0002211
type: UnificationXref
id: HMDB0002211
db: HMDB

Reference/ChEBI CHEBI 29034
type: UnificationXref
id: CHEBI:29034
db: ChEBI

Reference/HMDB HMDB0001361
type: UnificationXref
id: HMDB0001361
db: HMDB

Reference/ChemSpider 16522
type: UnificationXref
id: 16522
db: ChemSpider

Fe3+
type: SmallMoleculeReference
xref: Reference/PubChem-compound 29936
xref: Reference/ChemSpider 27815
xref: Reference/KEGG Compound C14819
xref: Reference/BioCyc CPD-10134
xref: Reference/HMDB HMDB0012943
xref: Reference/CAS 20074-52-6
xref: Reference/ChEBI CHEBI 29034
chemical formula: Fe
molecular weight: 55.93494
structure: ChemicalStructure/PW C008133

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

Reference/PubChem-compound 5353439
type: UnificationXref
id: 5353439
db: PubChem-compound

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

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

Bilirubin diglucuronide
type: SmallMolecule
cellular location: Cytosol
entity reference: Bilirubin diglucuronide

Compound/PW C008133 Mitochondrion/Stoichiometry/4.0
type: Stoichiometry
physical entity: Fe3+
stoichiometric coefficient: 4.0

ChemicalStructure/PW C000038
type: ChemicalStructure
structure format: SMILES

Reference/BioCyc FARNESYL-PP
type: UnificationXref
id: FARNESYL-PP
db: BioCyc

Transport10411
type: Transport
participant stoichiometry: Compound/PW C001799 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001799 Cytoplasm/Stoichiometry/1.0
right: Heme
spontaneous: false
left: Heme
name: Heme (→) Transport: Homo sapiens, Cell, Mitochondrion to Homo sapiens, Cell, Cytoplasm
conversion direction: LEFT_TO_RIGHT

Reference/CAS 630-08-0
type: UnificationXref
id: 630-08-0
db: CAS

ChemicalStructure/PW C001496
type: ChemicalStructure
structure format: SMILES

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

Ferritin, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q8N4E7
organism: Homo sapiens

Coproporphyrinogen III
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Coproporphyrinogen III

Reference/HMDB HMDB0001137
type: UnificationXref
id: HMDB0001137
db: HMDB

ChemicalStructure/PW C000163
type: ChemicalStructure
structure format: SMILES
structure data: CC1=C(CCC(O)=O)/C2=C/C3=N/C(=C\C4=C(C)C(C=C)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(C=C)=C4C)/C(C)=C3CCC(O)=O

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

ChemicalStructure/PW C000166
type: ChemicalStructure
structure format: SMILES
structure data: NCC1=C(CC(O)=O)C(CCC(O)=O)=CN1

Compound/PW C000974 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Coproporphyrinogen III
stoichiometric coefficient: 1.0

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

Protoheme IX farnesyltransferase, mitochondrial
type: ProteinReference
xref: Reference/UniProt Q12887
organism: Homo sapiens

Reference/BioCyc UROPORPHYRINOGEN-III
type: UnificationXref
id: UROPORPHYRINOGEN-III
db: BioCyc

Reference/KEGG Compound C05767
type: UnificationXref
id: C05767
db: KEGG Compound

Uroporphyrinogen decarboxylase
type: Protein
cellular location: Cytosol
entity reference: Uroporphyrinogen decarboxylase

Reference/PubChem-compound 121893
type: UnificationXref
id: 121893
db: PubChem-compound

Reference/CAS 58-98-0
type: UnificationXref
id: 58-98-0
db: CAS

SubPathwayInteraction639
type: Interaction
name: SubPathway639Reaction
name: SubPathwayReaction
participant: Glycine

Reference/KEGG Compound C05766
type: UnificationXref
id: C05766
db: KEGG Compound

Compound/PW C000887 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Hydroxymethylbilane
stoichiometric coefficient: 1.0

Reference/KEGG Compound C05769
type: UnificationXref
id: C05769
db: KEGG Compound

Heme
type: SmallMoleculeReference
xref: Reference/CAS 14875-96-8
xref: Reference/ChemSpider 24604415
xref: Reference/ChEBI CHEBI 17627
xref: Reference/KEGG Compound C00032
xref: Reference/BioCyc HEME A
xref: Reference/HMDB HMDB0003178
chemical formula: C34H32FeN4O4
molecular weight: 616.1773
structure: ChemicalStructure/PW C001799

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/KEGG Compound C05768
type: UnificationXref
id: C05768
db: KEGG Compound

Coproporphyrinogen III
type: SmallMolecule
cellular location: Cytosol
entity reference: Coproporphyrinogen III

Reference/KEGG Compound C05770
type: UnificationXref
id: C05770
db: KEGG Compound

Reference/HMDB HMDB0003325
type: UnificationXref
id: HMDB0003325
db: HMDB

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

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

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

Compound/PW C001051 Mitochondrion/Stoichiometry/2.0
type: Stoichiometry
physical entity: Hydrogen
stoichiometric coefficient: 2.0

Reference/KEGG Compound C01051
type: UnificationXref
id: C01051
db: KEGG Compound

ChemicalStructure/PW C003003
type: ChemicalStructure
structure format: SMILES

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

Reference/HMDB HMDB0001261
type: UnificationXref
id: HMDB0001261
db: HMDB

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

Reference/HMDB HMDB0000295
type: UnificationXref
id: HMDB0000295
db: HMDB

Reference/ChemSpider 5809
type: UnificationXref
id: 5809
db: ChemSpider

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

Reference/HMDB HMDB0000054
type: UnificationXref
id: HMDB0000054
db: HMDB

Compound/PW C000544 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Fe2+
stoichiometric coefficient: 1.0

Ferrochelatase, mitochondrial
type: ProteinReference
xref: Reference/UniProt P22830
organism: Homo sapiens

Delta-aminolevulinic acid dehydratase
type: ProteinReference
xref: Reference/UniProt P13716
organism: Homo sapiens

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

Compound/PW C000035 Cytosol/Stoichiometry/4.0
type: Stoichiometry
physical entity: Ammonia
stoichiometric coefficient: 4.0

Coproporphyrinogen I
type: SmallMoleculeReference
xref: Reference/BioCyc COPROPORPHYRINOGEN I
xref: Reference/ChemSpider 389645
xref: Reference/HMDB HMDB0002158
xref: Reference/CAS 31110-56-2
xref: Reference/ChEBI CHEBI 28607
xref: Reference/KEGG Compound C05768
xref: Reference/PubChem-compound 440776
chemical formula: C36H44N4O8
molecular weight: 660.3159
structure: ChemicalStructure/PW C001451

Protoporphyrinogen oxidase
type: Protein
entity reference: Protoporphyrinogen oxidase

Coproporphyrin III
type: SmallMoleculeReference
xref: Reference/KEGG Compound C05770
xref: Reference/CAS 14643-66-4
xref: Reference/HMDB HMDB0000570
xref: Reference/ChEBI CHEBI 27609
xref: Reference/ChemSpider 16736509
chemical formula: C36H38N4O8
molecular weight: 654.269
structure: ChemicalStructure/PW C000445

Reference/KEGG Compound C05787
type: UnificationXref
id: C05787
db: KEGG Compound

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

Reference/ChemSpider 995
type: UnificationXref
id: 995
db: ChemSpider

Reference/KEGG Compound C05791
type: UnificationXref
id: C05791
db: KEGG Compound

ReactionCatalysis1700
type: Catalysis
controller: Delta-aminolevulinic acid dehydratase
controlled: 4.2.1.24
control type: ACTIVATION

ReactionCatalysis1702
type: Catalysis
controller: Porphobilinogen deaminase
controlled: 2.5.1.61
control type: ACTIVATION

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

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

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

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

Reaction1911
type: BiochemicalReaction
participant stoichiometry: Compound/PW C003003 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000900 Mitochondrion/Stoichiometry/1.0
right: Heme A
spontaneous: false
left: Heme O
name: Heme O → Heme A
conversion direction: LEFT_TO_RIGHT

Reference/HMDB HMDB0001162
type: UnificationXref
id: HMDB0001162
db: HMDB

Reference/CAS 1867-62-5
type: UnificationXref
id: 1867-62-5
db: CAS

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

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

Reference/CAS 15438-31-0
type: UnificationXref
id: 15438-31-0
db: CAS

Farnesyl pyrophosphate
type: SmallMolecule
cellular location: Cytosol
entity reference: Farnesyl pyrophosphate

Compound/PW C001451 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Coproporphyrinogen I
stoichiometric coefficient: 1.0

Compound/PW C000445 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Coproporphyrin III
stoichiometric coefficient: 1.0

Compound/PW C000768 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Farnesyl pyrophosphate
stoichiometric coefficient: 1.0

Reference/BioCyc UROPORPHYRIN I
type: UnificationXref
id: UROPORPHYRIN_I
db: BioCyc

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

Reference/CAS 31110-56-2
type: UnificationXref
id: 31110-56-2
db: CAS

Reference/ChemSpider 25394
type: UnificationXref
id: 25394
db: ChemSpider

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

ReactionCatalysis1706
type: Catalysis
controller: Uroporphyrinogen-III synthase
controlled: 4.2.1.75
control type: ACTIVATION

Reference/KEGG Compound C01079
type: UnificationXref
id: C01079
db: KEGG Compound

Compound/PW C001316 Mitochondrion/Stoichiometry/2.0
type: Stoichiometry
physical entity: Carbon dioxide
stoichiometric coefficient: 2.0

ReactionCatalysis1708
type: Catalysis
controller: Uroporphyrinogen decarboxylase
controlled: 4.1.1.37
control type: ACTIVATION

ReactionCatalysis1709
type: Catalysis
controller: Uroporphyrinogen decarboxylase
controlled: 4.1.1.37
control type: ACTIVATION

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

Reference/KEGG Compound C03263
type: UnificationXref
id: C03263
db: KEGG Compound

ReactionCatalysis1710
type: Catalysis
controller: Coproporphyrinogen-III oxidase, mitochondrial
controlled: 1.3.3.3
control type: ACTIVATION

Reference/ChemSpider 10469486
type: UnificationXref
id: 10469486
db: ChemSpider

Compound/PW C002078 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Urobilinogen
stoichiometric coefficient: 1.0

2.5.1.-
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000768 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000900 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001799 Cytosol/Stoichiometry/1.0
right: Heme O
spontaneous: false
EC number: 2.5.1.-
left: Farnesyl pyrophosphate
left: Heme
name: Farnesyl pyrophosphate + Heme → Heme O
conversion direction: LEFT_TO_RIGHT

Reference/BioCyc COPROPORPHYRINOGEN III
type: UnificationXref
id: COPROPORPHYRINOGEN_III
db: BioCyc

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

Carbon monoxide
type: SmallMolecule
cellular location: Cytosol
entity reference: Carbon monoxide

ChemicalStructure/PW C001050
type: ChemicalStructure
structure format: SMILES
structure data: [C-]#[O+]

Reference/ChemSpider 16736727
type: UnificationXref
id: 16736727
db: ChemSpider

Reference/ChemSpider 16736725
type: UnificationXref
id: 16736725
db: ChemSpider

Cytochrome c oxidase assembly protein COX15 homolog
type: ProteinReference
xref: Reference/UniProt Q7KZN9
organism: Homo sapiens

ChemicalStructure/PW C000445
type: ChemicalStructure
structure format: SMILES
structure data: CC1=C(CCC(O)=O)/C2=C/C3=N/C(=C\C4=C(C)C(CCC(O)=O)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(C)=C4CCC(O)=O)/C(CCC(O)=O)=C3C

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

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

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

3.2.1.31
type: BiochemicalReaction
participant stoichiometry: Compound/PW C002078 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/Generic20 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001838 Cytosol/Stoichiometry/1.0
right: Alcohol
right: Urobilinogen
spontaneous: false
EC number: 3.2.1.31
left: Bilirubin diglucuronide
left: Water
name: Bilirubin diglucuronide + Water → Alcohol + Urobilinogen
conversion direction: LEFT_TO_RIGHT

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

Coproporphyrinogen I
type: SmallMolecule
cellular location: Cytosol
entity reference: Coproporphyrinogen I

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

TransportCatalysis1577
type: Catalysis
controller: Feline leukemia virus subgroup C receptor-related protein 2
controlled: Transport10411
control type: ACTIVATION

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

2.4.1.17
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000201 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000038 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000746 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001838 Cytosol/Stoichiometry/1.0
right: Uridine 5'-diphosphate
right: Bilirubin diglucuronide
spontaneous: false
EC number: 2.4.1.17
left: Bilirubin
left: Uridine diphosphate glucuronic acid
name: Bilirubin + Uridine diphosphate glucuronic acid → Bilirubin diglucuronide + Uridine 5'-diphosphate
conversion direction: LEFT_TO_RIGHT

Bilirubin
type: SmallMoleculeReference
xref: Reference/BioCyc BILIRUBIN
xref: Reference/ChemSpider 4444055
xref: Reference/PubChem-compound 5280352
xref: Reference/ChEBI CHEBI 16990
xref: Reference/CAS 635-65-4
xref: Reference/HMDB HMDB0000054
xref: Reference/KEGG Compound C00486
chemical formula: C33H36N4O6
molecular weight: 584.2635
structure: ChemicalStructure/PW C000038

Reference/CAS 2616-64-0
type: UnificationXref
id: 2616-64-0
db: CAS

Feline leukemia virus subgroup C receptor-related protein 2
type: Protein
entity reference: Feline leukemia virus subgroup C receptor-related protein 2

1.3.1.24
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000797 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000721 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000038 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001144 Cytosol/Stoichiometry/1.0
right: NADH
right: Bilirubin
spontaneous: false
EC number: 1.3.1.24
left: NAD
left: Biliverdin
name: Biliverdin + NAD → Bilirubin + NADH
conversion direction: LEFT_TO_RIGHT

Protoporphyrinogen oxidase
type: ProteinReference
xref: Reference/UniProt P50336

Beta-glucuronidase
type: Protein
cellular location: Cytosol
entity reference: Beta-glucuronidase

Protoporphyrin IX
type: SmallMolecule
entity reference: Protoporphyrin IX

ReactionCatalysis1699
type: Catalysis
controller: 5-aminolevulinate synthase, nonspecific, mitochondrial
controlled: 2.3.1.37
control type: ACTIVATION

Reference/ChemSpider 393270
type: UnificationXref
id: 393270
db: ChemSpider

Uroporphyrinogen I
type: SmallMoleculeReference
xref: Reference/ChemSpider 389644
xref: Reference/ChEBI CHEBI 28766
xref: Reference/BioCyc UROPORPHYRINOGEN-III
xref: Reference/KEGG Compound C05766
xref: Reference/CAS 1867-62-5
xref: Reference/HMDB HMDB0002211
xref: Reference/PubChem-compound 440775
chemical formula: C40H44N4O16
molecular weight: 836.2752
structure: ChemicalStructure/PW C001496

Reference/KEGG Compound C02191
type: UnificationXref
id: C02191
db: KEGG Compound

Compound/PW C001050 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Carbon monoxide
stoichiometric coefficient: 1.0

Reference/PubChem-compound 5280352
type: UnificationXref
id: 5280352
db: PubChem-compound

1.14.99.3
type: BiochemicalReaction
participant stoichiometry: Compound/Generic66 Cytosol/Stoichiometry/3.0
participant stoichiometry: Compound/PW C000544 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001050 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001799 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000797 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/3.0
participant stoichiometry: Compound/PW C001065 Cytosol/Stoichiometry/3.0
participant stoichiometry: Compound/Generic55 Cytosol/Stoichiometry/3.0
right: A
right: Fe2+
right: Biliverdin
right: Carbon monoxide
right: Water
spontaneous: false
EC number: 1.14.99.3
left: Oxygen
left: AH2
left: Heme
name: 3 AH2 + Heme + 3 Oxygen → 3 A + Biliverdin + Carbon monoxide + Fe2+ + 3 Water
conversion direction: LEFT_TO_RIGHT

Reference/ChemSpider 3571849
type: UnificationXref
id: 3571849
db: ChemSpider

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

Porphobilinogen
type: SmallMoleculeReference
xref: Reference/ChemSpider 995
xref: Reference/KEGG Compound C00931
xref: Reference/HMDB HMDB0000245
xref: Reference/CAS 487-90-1
xref: Reference/ChEBI CHEBI 17381
xref: Reference/BioCyc PORPHOBILINOGEN
xref: Reference/PubChem-compound 1021
chemical formula: C10H14N2O4
molecular weight: 226.09535
structure: ChemicalStructure/PW C000166

Compound/PW C001420 Mitochondrion/Stoichiometry/2.0
type: Stoichiometry
physical entity: Water
stoichiometric coefficient: 2.0

Protoporphyrin IX
type: SmallMoleculeReference
xref: Reference/CAS 553-12-8
xref: Reference/KEGG Compound C02191
xref: Reference/BioCyc PROTOPORPHYRIN IX
xref: Reference/ChemSpider 10469486
xref: Reference/ChEBI CHEBI 15430
xref: Reference/HMDB HMDB0000241
chemical formula: C34H34N4O4
molecular weight: 562.258
structure: ChemicalStructure/PW C000163

Reference/ChemSpider 16736701
type: UnificationXref
id: 16736701
db: ChemSpider

ChemicalStructure/PW C000544
type: ChemicalStructure
structure format: SMILES
structure data: [Fe++]

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

Reference/ChemSpider 275
type: UnificationXref
id: 275
db: ChemSpider

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

Reference/BioCyc Ferric-Hydroxamate-Complexes
type: UnificationXref
id: Ferric-Hydroxamate-Complexes
db: BioCyc

Reference/ChemSpider 4510089
type: UnificationXref
id: 4510089
db: ChemSpider

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

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

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

4.2.1.75
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000854 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000887 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/1.0
right: Uroporphyrinogen III
right: Water
spontaneous: false
EC number: 4.2.1.75
left: Hydroxymethylbilane
name: Hydroxymethylbilane → Uroporphyrinogen III + Water
conversion direction: LEFT_TO_RIGHT

ChemicalStructure/PW C000797
type: ChemicalStructure
structure format: SMILES

Reference/PubChem-compound 1021
type: UnificationXref
id: 1021
db: PubChem-compound

Reference/CAS 137397-56-9
type: UnificationXref
id: 137397-56-9
db: CAS

Reference/CAS 7412-77-3
type: UnificationXref
id: 7412-77-3
db: CAS

ChemicalStructure/PW C000201
type: ChemicalStructure
structure format: SMILES

Reference/UniProt P36551
type: UnificationXref
id: P36551
db: UniProt

Coproporphyrin III
type: SmallMolecule
cellular location: Cytosol
entity reference: Coproporphyrin III

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

Compound/PW C001838 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Bilirubin diglucuronide
stoichiometric coefficient: 1.0

Compound/PW C000854 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Uroporphyrinogen III
stoichiometric coefficient: 1.0

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

Carbon dioxide
type: SmallMolecule
cellular location: Cytosol
entity reference: Carbon dioxide

Reference/PubChem-compound 27284
type: UnificationXref
id: 27284
db: PubChem-compound

Reference/UniProt P08397
type: UnificationXref
id: P08397
db: UniProt

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

Reference/PubChem-compound 321
type: UnificationXref
id: 321
db: PubChem-compound

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

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

Reference/ChemSpider 108741
type: UnificationXref
id: 108741
db: ChemSpider

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

ReactionCatalysis1766
type: Catalysis
controller: Biliverdin reductase A
controlled: 1.3.1.24
control type: ACTIVATION

Reference/ChEBI CHEBI 24480
type: UnificationXref
id: CHEBI:24480
db: ChEBI

ReactionCatalysis1765
type: Catalysis
controller: Heme oxygenase 1
controlled: 1.14.99.3
control type: ACTIVATION

ReactionCatalysis1767
type: Catalysis
controller: UDP-glucuronosyltransferase 2B11
controlled: 2.4.1.17
control type: ACTIVATION

Oxygen
type: SmallMolecule
entity reference: Oxygen

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

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

Fe2+
type: SmallMolecule
cellular location: Cytosol
entity reference: Fe2+

Reference/HMDB HMDB0000570
type: UnificationXref
id: HMDB0000570
db: HMDB

Reference/HMDB HMDB0000692
type: UnificationXref
id: HMDB0000692
db: HMDB

Compound/PW C001316 Cytosol/Stoichiometry/4.0
type: Stoichiometry
physical entity: Carbon dioxide
stoichiometric coefficient: 4.0

Compound/PW C001065 Cytosol/Stoichiometry/3.0
type: Stoichiometry
physical entity: Oxygen
stoichiometric coefficient: 3.0

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

Compound/PW C000038 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Bilirubin
stoichiometric coefficient: 1.0

Reference/CAS 635-65-4
type: UnificationXref
id: 635-65-4
db: CAS

Compound/PW C001799 Cytoplasm/Stoichiometry/1.0
type: Stoichiometry
physical entity: Heme
stoichiometric coefficient: 1.0

Compound/PW C001065 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Oxygen
stoichiometric coefficient: 1.0

Reference/UniProt Q8N4E7
type: UnificationXref
id: Q8N4E7
db: UniProt

Heme O
type: SmallMolecule
cellular location: Cytosol
entity reference: Heme O

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

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

Reference/PubChem-compound 17473
type: UnificationXref
id: 17473
db: PubChem-compound

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

Compound/PW C000502 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Coproporphyrin I
stoichiometric coefficient: 1.0

Glycine and Serine Metabolism
type: Pathway
pathway order: SubPathwayInteractionSteps/SubPathway639
pathway component: SubPathwayInteraction639
xref: Reference/PathWhiz PW000157
xref: Reference/SMPDB SMP0000004
name: SubPathway

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

Carbon monoxide
type: SmallMoleculeReference
xref: Reference/KEGG Compound C00237
xref: Reference/ChemSpider 275
xref: Reference/PubChem-compound 281
xref: Reference/HMDB HMDB0001361
xref: Reference/ChEBI CHEBI 17245
xref: Reference/CAS 630-08-0
chemical formula: CO
molecular weight: 27.994915
structure: ChemicalStructure/PW C001050

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

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

Reference/KEGG Compound C00931
type: UnificationXref
id: C00931
db: KEGG Compound

Reference/ChemSpider 4444055
type: UnificationXref
id: 4444055
db: ChemSpider

Reaction1879
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001496 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000887 Cytosol/Stoichiometry/1.0
right: Uroporphyrinogen I
spontaneous: false
left: Hydroxymethylbilane
name: Hydroxymethylbilane → Uroporphyrinogen I
conversion direction: LEFT_TO_RIGHT

Compound/PW C001496 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Uroporphyrinogen I
stoichiometric coefficient: 1.0

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

ChemicalStructure/PW C001799
type: ChemicalStructure
structure format: SMILES

Reference/ChEBI CHEBI 27609
type: UnificationXref
id: CHEBI:27609
db: ChEBI

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

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

Compound/PW C000544 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Fe2+
stoichiometric coefficient: 1.0

Reaction1877
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000854 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000732 Cytosol/Stoichiometry/1.0
right: Uroporphyrin III
spontaneous: false
left: Uroporphyrinogen III
name: Uroporphyrinogen III → Uroporphyrin III
conversion direction: LEFT_TO_RIGHT

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

Compound/PW C001799 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Heme
stoichiometric coefficient: 1.0

Reference/CAS 17459-92-6
type: UnificationXref
id: 17459-92-6
db: CAS

2.5.1.61
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000035 Cytosol/Stoichiometry/4.0
participant stoichiometry: Compound/PW C000166 Cytosol/Stoichiometry/4.0
participant stoichiometry: Compound/PW C000887 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/1.0
right: Ammonia
right: Hydroxymethylbilane
spontaneous: false
EC number: 2.5.1.61
left: Porphobilinogen
left: Water
name: 4 Porphobilinogen + Water → 4 Ammonia + Hydroxymethylbilane
conversion direction: LEFT_TO_RIGHT

2.3.1.37
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001316 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000894 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000808 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000078 Cytosol/Stoichiometry/1.0
right: 5-Aminolevulinic acid
right: Carbon dioxide
spontaneous: false
EC number: 2.3.1.37
left: Glycine
left: Succinyl-CoA
name: Glycine + Succinyl-CoA → 5-Aminolevulinic acid + Carbon dioxide
conversion direction: LEFT_TO_RIGHT

Reference/ChemSpider 16736509
type: UnificationXref
id: 16736509
db: ChemSpider

4.2.1.24
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000894 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000166 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001420 Cytosol/Stoichiometry/2.0
right: Porphobilinogen
right: Water
spontaneous: false
EC number: 4.2.1.24
left: 5-Aminolevulinic acid
name: 5-Aminolevulinic acid → Porphobilinogen + 2 Water
conversion direction: LEFT_TO_RIGHT

ReactionCatalysis1783
type: Catalysis
controller: Beta-glucuronidase
controlled: 3.2.1.31
control type: ACTIVATION

Compound/PW C000166 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Porphobilinogen
stoichiometric coefficient: 1.0

Compound/PW C000163 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Protoporphyrin IX
stoichiometric coefficient: 1.0

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

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

ChemicalStructure/PW C001451
type: ChemicalStructure
structure format: SMILES

Compound/PW C000974 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Coproporphyrinogen III
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0000245
type: UnificationXref
id: HMDB0000245
db: HMDB

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

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

Reference/HMDB HMDB0006901
type: UnificationXref
id: HMDB0006901
db: HMDB

Compound/PW C001420 Cytosol/Stoichiometry/3.0
type: Stoichiometry
physical entity: Water
stoichiometric coefficient: 3.0

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

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

Reference/HMDB HMDB0000241
type: UnificationXref
id: HMDB0000241
db: HMDB

Heme O
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Heme O

Reference/ChEBI CHEBI 17627
type: UnificationXref
id: CHEBI:17627
db: ChEBI

Reference/UniProt P08236
type: UnificationXref
id: P08236
db: UniProt

A
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 13193

ChemicalStructure/PW C000721
type: ChemicalStructure
structure format: SMILES

Compound/Generic55 Cytosol/Stoichiometry/3.0
type: Stoichiometry
physical entity: AH2
stoichiometric coefficient: 3.0

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

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

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

Reference/PubChem-compound 440784
type: UnificationXref
id: 440784
db: PubChem-compound

1.3.3.4
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000863/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000163/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001783/Stoichiometry/3.0
participant stoichiometry: Compound/PW C001065/Stoichiometry/3.0
right: Protoporphyrin IX
right: Hydrogen peroxide
spontaneous: false
EC number: 1.3.3.4
left: Oxygen
left: Protoporphyrinogen IX
name: 3 Oxygen + Protoporphyrinogen IX → 3 Hydrogen peroxide + Protoporphyrin IX
conversion direction: LEFT_TO_RIGHT

Reference/ChEBI CHEBI 28766
type: UnificationXref
id: CHEBI:28766
db: ChEBI

Reference/CAS 14643-66-4
type: UnificationXref
id: 14643-66-4
db: CAS

Reference/CAS 14875-96-8
type: UnificationXref
id: 14875-96-8
db: CAS

Reference/ChEBI CHEBI 15439
type: UnificationXref
id: CHEBI:15439
db: ChEBI

Reference/SMPDB SMP0000004
type: UnificationXref
id: SMP0000004
db: SMPDB

1.16.3.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001065 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001051 Mitochondrion/Stoichiometry/4.0
participant stoichiometry: Compound/PW C000544 Mitochondrion/Stoichiometry/4.0
participant stoichiometry: Compound/PW C008133 Mitochondrion/Stoichiometry/4.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/2.0
right: Water
right: Fe3+
spontaneous: false
EC number: 1.16.3.1
left: Fe2+
left: Hydrogen
left: Oxygen
name: 4 Fe2+ + 4 Hydrogen + Oxygen ← 4 Fe3+ + 2 Water
conversion direction: RIGHT_TO_LEFT

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

4.99.1.1
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000544 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001051 Mitochondrion/Stoichiometry/2.0
participant stoichiometry: Compound/PW C000163 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001799 Mitochondrion/Stoichiometry/1.0
right: Hydrogen
right: Heme
spontaneous: false
EC number: 4.99.1.1
left: Fe2+
left: Protoporphyrin IX
name: Fe2+ + Protoporphyrin IX → Heme + 2 Hydrogen
conversion direction: LEFT_TO_RIGHT

Reference/UniProt P13716
type: UnificationXref
id: P13716
db: UniProt

Delta-aminolevulinic acid dehydratase
type: Protein
cellular location: Cytosol
entity reference: Delta-aminolevulinic acid dehydratase

ChemicalStructure/PW C000854
type: ChemicalStructure
structure format: SMILES

AH2
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 17499

ChemicalStructure/PW C000732
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CCC1=C(CC(O)=O)/C2=C/C3=N/C(=C\C4=C(CCC(O)=O)C(CC(O)=O)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(CCC(O)=O)=C4CC(O)=O)/C(CC(O)=O)=C3CCC(O)=O

Reference/HMDB HMDB0000936
type: UnificationXref
id: HMDB0000936
db: HMDB

ChemicalStructure/PW C000974
type: ChemicalStructure
structure format: SMILES

Reference/HMDB HMDB0000935
type: UnificationXref
id: HMDB0000935
db: HMDB

Reference/PubChem-compound 440776
type: UnificationXref
id: 440776
db: PubChem-compound

Reference/PubChem-compound 440775
type: UnificationXref
id: 440775
db: PubChem-compound

Carbon dioxide
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Carbon dioxide

4.1.1.37
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001451 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001496 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Cytosol/Stoichiometry/4.0
right: Carbon dioxide
right: Coproporphyrinogen I
spontaneous: false
EC number: 4.1.1.37
left: Uroporphyrinogen I
name: Uroporphyrinogen I → 4 Carbon dioxide + Coproporphyrinogen I
conversion direction: LEFT_TO_RIGHT

1.3.3.3
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000863 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000974 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001065 Mitochondrion/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001051 Mitochondrion/Stoichiometry/2.0
participant stoichiometry: Compound/PW C001316 Mitochondrion/Stoichiometry/2.0
participant stoichiometry: Compound/PW C001420 Mitochondrion/Stoichiometry/2.0
right: Carbon dioxide
right: Water
right: Protoporphyrinogen IX
spontaneous: false
EC number: 1.3.3.3
left: Coproporphyrinogen III
left: Hydrogen
left: Oxygen
name: Coproporphyrinogen III + 2 Hydrogen + Oxygen → 2 Carbon dioxide + Protoporphyrinogen IX + 2 Water
conversion direction: LEFT_TO_RIGHT

Fe3+
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Fe3+

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

Compound/PW C001420 Cytosol/Stoichiometry/2.0
type: Stoichiometry
physical entity: Water
stoichiometric coefficient: 2.0

Reaction1886
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000445 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000974 Cytosol/Stoichiometry/1.0
right: Coproporphyrin III
spontaneous: false
left: Coproporphyrinogen III
name: Coproporphyrinogen III → Coproporphyrin III
conversion direction: LEFT_TO_RIGHT

Reaction1884
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000747 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001496 Cytosol/Stoichiometry/1.0
right: Uroporphyrin I
spontaneous: false
left: Uroporphyrinogen I
name: Uroporphyrinogen I → Uroporphyrin I
conversion direction: LEFT_TO_RIGHT

4.1.1.37
type: BiochemicalReaction
participant stoichiometry: Compound/PW C000854 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C001316 Cytosol/Stoichiometry/4.0
participant stoichiometry: Compound/PW C000974 Cytosol/Stoichiometry/1.0
right: Carbon dioxide
right: Coproporphyrinogen III
spontaneous: false
EC number: 4.1.1.37
left: Uroporphyrinogen III
name: Uroporphyrinogen III → 4 Carbon dioxide + Coproporphyrinogen III
conversion direction: LEFT_TO_RIGHT

Reference/CAS 57560-10-8
type: UnificationXref
id: 57560-10-8
db: CAS

Reaction1883
type: BiochemicalReaction
participant stoichiometry: Compound/PW C001451 Cytosol/Stoichiometry/1.0
participant stoichiometry: Compound/PW C000502 Cytosol/Stoichiometry/1.0
right: Coproporphyrin I
spontaneous: false
left: Coproporphyrinogen I
name: Coproporphyrinogen I → Coproporphyrin I
conversion direction: LEFT_TO_RIGHT

Reference/PubChem-compound 5459911
type: UnificationXref
id: 5459911
db: PubChem-compound

Compound/PW C001783/Stoichiometry/3.0
type: Stoichiometry
physical entity: Hydrogen peroxide
stoichiometric coefficient: 3.0

Reference/CAS 1976-85-8
type: UnificationXref
id: 1976-85-8
db: CAS

Homo sapiens
type: BioSource
xref: Reference/TAXONOMY 9606

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

Protoheme IX farnesyltransferase, mitochondrial
type: Protein
cellular location: Cytosol
entity reference: Protoheme IX farnesyltransferase, mitochondrial

Reference/ChEBI CHEBI 16990
type: UnificationXref
id: CHEBI:16990
db: ChEBI

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

Uridine diphosphate glucuronic acid
type: SmallMolecule
cellular location: Cytosol
entity reference: Uridine diphosphate glucuronic acid

Protoporphyrin IX
type: SmallMolecule
cellular location: Mitochondrion
entity reference: Protoporphyrin IX

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

Heme A
type: SmallMoleculeReference
xref: Reference/KEGG Compound C15670
xref: Reference/HMDB HMDB0006901
xref: Reference/CAS 57560-10-8
chemical formula: C49H56FeN4O6
molecular weight: 852.3549
structure: ChemicalStructure/PW C003003

Compound/Generic20 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Alcohol
stoichiometric coefficient: 1.0

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

Reference/ChEBI CHEBI 15436
type: UnificationXref
id: CHEBI:15436
db: ChEBI

Reference/ChEBI CHEBI 16645
type: UnificationXref
id: CHEBI:16645
db: ChEBI

Reference/ChEBI CHEBI 15435
type: UnificationXref
id: CHEBI:15435
db: ChEBI

Reference/HMDB HMDB0012943
type: UnificationXref
id: HMDB0012943
db: HMDB

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

Reference/ChEBI CHEBI 15437
type: UnificationXref
id: CHEBI:15437
db: ChEBI

Reference/CAS 487-90-1
type: UnificationXref
id: 487-90-1
db: CAS

Reference/ChEBI CHEBI 28607
type: UnificationXref
id: CHEBI:28607
db: ChEBI

Reference/BioCyc HEME O
type: UnificationXref
id: HEME_O
db: BioCyc

Reference/KEGG Compound C00500
type: UnificationXref
id: C00500
db: KEGG Compound

Protoporphyrin IX
type: SmallMolecule
cellular location: Mitochondrial Inner Membrane
entity reference: Protoporphyrin IX

Reference/ChEBI CHEBI 15430
type: UnificationXref
id: CHEBI:15430
db: ChEBI

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

Compound/PW C000747 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Uroporphyrin I
stoichiometric coefficient: 1.0

Reference/HMDB HMDB0000961
type: UnificationXref
id: HMDB0000961
db: HMDB

Reference/UniProt Q9UPI3
type: UnificationXref
id: Q9UPI3
db: UniProt

Cytochrome c oxidase assembly protein COX15 homolog
type: Protein
cellular location: Mitochondrion
entity reference: Cytochrome c oxidase assembly protein COX15 homolog

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

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

Reference/BioCyc HEME A
type: UnificationXref
id: HEME_A
db: BioCyc

Porphyria Variegata (PV)
type: Pathway
pathway order: PathwayEnzymeReaction/2281
pathway order: PathwayEnzymeReaction/211575
pathway order: PathwayEnzymeReaction/1193299
pathway order: PathwayEnzymeReaction/2286
pathway order: PathwayEnzymeReaction/2342
pathway order: PathwaySubPathway/639
pathway order: PathwayEnzymeReaction/2387
pathway order: PathwayEnzymeReaction/1193296
pathway order: PathwayEnzymeReaction/2282
pathway order: PathwayEnzymeReaction/2283
pathway order: PathwayEnzymeReaction/2284
pathway order: PathwayEnzymeReaction/2279
pathway order: PathwayEnzymeReaction/2312
pathway order: PathwayTransport/1193298
pathway order: PathwayEnzymeReaction/2391
pathway order: PathwayTransport/2130264
pathway order: PathwayTransport/2310
pathway order: PathwayEnzymeReaction/2392
pathway order: PathwayEnzymeReaction/2393
pathway order: PathwayEnzymeReaction/2305
pathway order: PathwayEnzymeReaction/2327
pathway order: PathwayEnzymeReaction/2306
pathway order: PathwayEnzymeReaction/2302
pathway order: PathwayEnzymeReaction/2303
pathway order: PathwayEnzymeReaction/2347
pathway component: Transport178
pathway component: Reaction1879
pathway component: ReactionCatalysis1717
pathway component: ReactionCatalysis1716
pathway component: ReactionCatalysis1718
pathway component: ReactionCatalysis1741
pathway component: 1.3.3.4
pathway component: ReactionCatalysis1700
pathway component: ReactionCatalysis1766
pathway component: ReactionCatalysis1765
pathway component: ReactionCatalysis1702
pathway component: ReactionCatalysis1767
pathway component: ReactionCatalysis1726
pathway component: Reaction1877
pathway component: 3.2.1.31
pathway component: Reaction1911
pathway component: 1.16.3.1
pathway component: 2.5.1.61
pathway component: 4.99.1.1
pathway component: 2.3.1.37
pathway component: 4.2.1.24
pathway component: ReactionCatalysis1783
pathway component: TransportCatalysis1577
pathway component: 4.2.1.75
pathway component: 2.4.1.17
pathway component: 1.3.1.24
pathway component: Transport1220
pathway component: ReactionCatalysis1706
pathway component: ReactionCatalysis1708
pathway component: ReactionCatalysis1709
pathway component: Transport10411
pathway component: 4.1.1.37
pathway component: ReactionCatalysis1699
pathway component: ReactionCatalysis1710
pathway component: 1.14.99.3
pathway component: 1.3.3.3
pathway component: 2.5.1.-
pathway component: Reaction1886
pathway component: Reaction1884
pathway component: Glycine and Serine Metabolism
pathway component: Reaction1883
xref: Reference/PathWhiz PW000175
xref: Reference/SMPDB SMP0000346
organism: Homo sapiens

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

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

Coproporphyrinogen-III oxidase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Coproporphyrinogen-III oxidase, mitochondrial

Heme O
type: SmallMoleculeReference
xref: Reference/CAS 137397-56-9
xref: Reference/ChEBI CHEBI 24480
xref: Reference/KEGG Compound C15672
xref: Reference/BioCyc HEME O
xref: Reference/ChemSpider 3571849
xref: Reference/HMDB HMDB0001162
chemical formula: C49H58FeN4O5
molecular weight: 838.3757
structure: ChemicalStructure/PW C000900

Reference/UniProt P06132
type: UnificationXref
id: P06132
db: UniProt

Reference/UniProt Q7KZN9
type: UnificationXref
id: Q7KZN9
db: UniProt

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

Reference/UniProt P53004
type: UnificationXref
id: P53004
db: UniProt

Reference/ChemSpider 4573640
type: UnificationXref
id: 4573640
db: ChemSpider

Uroporphyrin I
type: SmallMolecule
cellular location: Cytosol
entity reference: Uroporphyrin I

ChemicalStructure/PW C000887
type: ChemicalStructure
structure format: SMILES

Reference/PubChem-compound 1179
type: UnificationXref
id: 1179
db: PubChem-compound

Reference/PubChem-compound 281
type: UnificationXref
id: 281
db: PubChem-compound

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

ChemicalStructure/PW C000768
type: ChemicalStructure
structure format: SMILES

Alcohol
type: SmallMoleculeReference
xref: Reference/ChEBI CHEBI 30879

Reference/ChEBI CHEBI 30879
type: UnificationXref
id: CHEBI:30879
db: ChEBI

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

Compound/PW C001065/Stoichiometry/3.0
type: Stoichiometry
physical entity: Oxygen
stoichiometric coefficient: 3.0

ChemicalStructure/PW C008133
type: ChemicalStructure
structure format: SMILES
structure data: [Fe+3]

Compound/PW C001799 Mitochondrion/Stoichiometry/1.0
type: Stoichiometry
physical entity: Heme
stoichiometric coefficient: 1.0

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

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

Ferrochelatase, mitochondrial
type: Protein
cellular location: Mitochondrion
entity reference: Ferrochelatase, mitochondrial

Reference/CAS 20074-52-6
type: UnificationXref
id: 20074-52-6
db: CAS

Reference/ChEBI CHEBI 13193
type: UnificationXref
id: CHEBI:13193
db: ChEBI

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

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

Biliverdin reductase A
type: Protein
cellular location: Cytosol
entity reference: Biliverdin reductase A

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

Reference/BioCyc PROTOPORPHYRIN IX
type: UnificationXref
id: PROTOPORPHYRIN_IX
db: BioCyc

Heme oxygenase 1
type: ProteinReference
xref: Reference/UniProt P09601
organism: Homo sapiens

Reference/ChEBI CHEBI 17407
type: UnificationXref
id: CHEBI:17407
db: ChEBI

Reference/SMPDB SMP0000346
type: UnificationXref
id: SMP0000346
db: SMPDB

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

ChemicalStructure/PW C000502
type: ChemicalStructure
structure format: SMILES
structure data: CC1=C(CCC(O)=O)/C2=C/C3=N/C(=C\C4=C(C)C(CCC(O)=O)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(CCC(O)=O)=C4C)/C(CCC(O)=O)=C3C

ChemicalStructure/PW C000863
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C001838
type: ChemicalStructure
structure format: SMILES

ChemicalStructure/PW C000747
type: ChemicalStructure
structure format: SMILES
structure data: OC(=O)CCC1=C(CC(O)=O)/C2=C/C3=N/C(=C\C4=C(CCC(O)=O)C(CC(O)=O)=C(N4)/C=C4\N=C(\C=C\1/N\2)C(CC(O)=O)=C4CCC(O)=O)/C(CC(O)=O)=C3CCC(O)=O

ChemicalStructure/PW C000746
type: ChemicalStructure
structure format: SMILES

Urobilinogen
type: SmallMoleculeReference
xref: Reference/KEGG Compound C05791
xref: Reference/PubChem-compound 440784
xref: Reference/CAS 17208-65-0
xref: Reference/ChemSpider 389649
xref: Reference/HMDB HMDB0004158
chemical formula: C33H42N4O6
molecular weight: 590.3104
structure: ChemicalStructure/PW C002078

Reference/KEGG Compound C14819
type: UnificationXref
id: C14819
db: KEGG Compound

Compound/PW C000900 Cytosol/Stoichiometry/1.0
type: Stoichiometry
physical entity: Heme O
stoichiometric coefficient: 1.0

Reference/KEGG Compound C14818
type: UnificationXref
id: C14818
db: KEGG Compound

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

Reference/ChEBI CHEBI 28421
type: UnificationXref
id: CHEBI:28421
db: ChEBI

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

Reference/ChemSpider 24604415
type: UnificationXref
id: 24604415
db: ChemSpider

Reference/ChEBI CHEBI 17659
type: UnificationXref
id: CHEBI:17659
db: ChEBI

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

Compound/PW C000163 Mitochondrial Matrix/Stoichiometry/1.0
type: Stoichiometry
physical entity: Protoporphyrin IX
stoichiometric coefficient: 1.0

Reference/CAS 2624-63-7
type: UnificationXref
id: 2624-63-7
db: CAS

Reference/HMDB HMDB0000643
type: UnificationXref
id: HMDB0000643
db: HMDB