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HUMAN:HEM6

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Species (Taxon ID) Homo sapiens (Human). (9606)
Gene Name(s) CPOX (synonyms: CPO, CPX)
Protein Name(s) Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial

COX Coprogen oxidase Coproporphyrinogenase

External Links
UniProt P36551
EMBL Z34531
Z34803
Z34804
Z34805
Z34806
Z34807
Z34808
AK290140
AK299692
AK223481
CH471052
BC017210
BC023551
BC023554
D16611
Z28409
CCDS CCDS2932.1
PIR I52444
RefSeq NP_000088.3
UniGene Hs.476982
PDB 2AEX
PDBsum 2AEX
ProteinModelPortal P36551
SMR P36551
BioGrid 107763
IntAct P36551
STRING 9606.ENSP00000264193
ChEMBL CHEMBL1681618
PhosphoSite P36551
DMDM 67476671
MaxQB P36551
PaxDb P36551
PeptideAtlas P36551
PRIDE P36551
Ensembl ENST00000264193
GeneID 1371
KEGG hsa:1371
UCSC uc003dsx.3
CTD 1371
GeneCards GC03M098239
GeneReviews CPOX
HGNC HGNC:2321
HPA HPA015736
HPA054448
MIM 121300
612732
neXtProt NX_P36551
Orphanet 79273
PharmGKB PA134979958
eggNOG COG0408
GeneTree ENSGT00390000017311
HOGENOM HOG000262768
HOVERGEN HBG051897
InParanoid P36551
KO K00228
OMA QRPEAKG
PhylomeDB P36551
TreeFam TF300703
BioCyc MetaCyc:HS01369-MONOMER
BRENDA 1.3.3.3
Reactome REACT_9465
UniPathway UPA00251
EvolutionaryTrace P36551
GeneWiki Coproporphyrinogen_III_oxidase
GenomeRNAi 1371
NextBio 35474630
PRO PR:P36551
Proteomes UP000005640
Bgee P36551
CleanEx HS_CPO
HS_CPOX
ExpressionAtlas P36551
Genevestigator P36551
GO GO:0005743
GO:0005758
GO:0005739
GO:0004109
GO:0042803
GO:0005212
GO:0006783
GO:0006779
GO:0006778
GO:0006782
GO:0046685
GO:0017085
GO:0010039
GO:0010288
GO:0051597
GO:0044281
Gene3D 3.40.1500.10
InterPro IPR001260
IPR018375
PANTHER PTHR10755
Pfam PF01218
PRINTS PR00073
SUPFAM SSF102886
PROSITE PS01021

Annotations

Qualifier GO ID GO term name Reference ECO ID ECO term name with/from Aspect Extension Notes Status

enables

GO:0042803

protein homodimerization activity

PMID:16176984[1]

ECO:0000314

direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

involved_in

GO:0019353

protoporphyrinogen IX biosynthetic process from glutamate

PMID:21873635[2]

ECO:0000318

biological aspect of ancestor evidence used in manual assertion

EcoGene:EG12189
PANTHER:PTN000079415

P

Seeded From UniProt

complete

part_of

GO:0005829

cytosol

PMID:21873635[2]

ECO:0000318

biological aspect of ancestor evidence used in manual assertion

PANTHER:PTN000079415
SGD:S000002451
UniProtKB:P36551
UniProtKB:Q8IHU1

C

Seeded From UniProt

complete

part_of

GO:0005737

cytoplasm

PMID:21873635[2]

ECO:0000318

biological aspect of ancestor evidence used in manual assertion

EcoGene:EG12189
PANTHER:PTN000079415

C

Seeded From UniProt

complete

enables

GO:0004109

coproporphyrinogen oxidase activity

PMID:21873635[2]

ECO:0000318

biological aspect of ancestor evidence used in manual assertion

EcoGene:EG12189
MGI:MGI:104841
PANTHER:PTN000079415
RGD:1311817
SGD:S000002451
TAIR:locus:2825062
UniProtKB:P43898
UniProtKB:P72848
UniProtKB:Q8IHU1

F

Seeded From UniProt

complete

part_of

GO:0005829

cytosol

GO_REF:0000052

ECO:0000314

direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

part_of

GO:0005739

mitochondrion

GO_REF:0000052

ECO:0000314

direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

part_of

GO:0016020

membrane

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:P36552
ensembl:ENSMUSP00000055455

C

Seeded From UniProt

complete

involved_in

GO:0006783

heme biosynthetic process

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:P36552
ensembl:ENSMUSP00000055455

P

Seeded From UniProt

complete

part_of

GO:0005739

mitochondrion

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:P36552
ensembl:ENSMUSP00000055455

C

Seeded From UniProt

complete

enables

GO:0005212

structural constituent of eye lens

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:P36552
ensembl:ENSMUSP00000055455

F

Seeded From UniProt

complete

enables

GO:0004109

coproporphyrinogen oxidase activity

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:P36552
ensembl:ENSMUSP00000055455

F

Seeded From UniProt

complete

involved_in

GO:0051597

response to methylmercury

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q3B7D0
ensembl:ENSRNOP00000002257

P

Seeded From UniProt

complete

involved_in

GO:0046685

response to arsenic-containing substance

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q3B7D0
ensembl:ENSRNOP00000002257

P

Seeded From UniProt

complete

enables

GO:0042802

identical protein binding

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q3B7D0
ensembl:ENSRNOP00000002257

F

Seeded From UniProt

complete

involved_in

GO:0017085

response to insecticide

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q3B7D0
ensembl:ENSRNOP00000002257

P

Seeded From UniProt

complete

involved_in

GO:0010288

response to lead ion

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q3B7D0
ensembl:ENSRNOP00000002257

P

Seeded From UniProt

complete

involved_in

GO:0010039

response to iron ion

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q3B7D0
ensembl:ENSRNOP00000002257

P

Seeded From UniProt

complete

involved_in

GO:0010035

response to inorganic substance

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q3B7D0
ensembl:ENSRNOP00000002257

P

Seeded From UniProt

complete

involved_in

GO:0006783

heme biosynthetic process

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q3B7D0
ensembl:ENSRNOP00000002257

P

Seeded From UniProt

complete

involved_in

GO:0006782

protoporphyrinogen IX biosynthetic process

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q3B7D0
ensembl:ENSRNOP00000002257

P

Seeded From UniProt

complete

part_of

GO:0005758

mitochondrial intermembrane space

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q3B7D0
ensembl:ENSRNOP00000002257

C

Seeded From UniProt

complete

part_of

GO:0005743

mitochondrial inner membrane

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q3B7D0
ensembl:ENSRNOP00000002257

C

Seeded From UniProt

complete

part_of

GO:0005739

mitochondrion

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q3B7D0
ensembl:ENSRNOP00000002257

C

Seeded From UniProt

complete

enables

GO:0004109

coproporphyrinogen oxidase activity

GO_REF:0000107

ECO:0000265

sequence orthology evidence used in automatic assertion

UniProtKB:Q3B7D0
ensembl:ENSRNOP00000002257

F

Seeded From UniProt

complete

enables

GO:0004109

coproporphyrinogen oxidase activity

GO_REF:0000002

ECO:0000256

match to sequence model evidence used in automatic assertion

InterPro:IPR001260
InterPro:IPR018375
InterPro:IPR036406

F

Seeded From UniProt

complete

involved_in

GO:0006779

porphyrin-containing compound biosynthetic process

GO_REF:0000002

ECO:0000256

match to sequence model evidence used in automatic assertion

InterPro:IPR001260
InterPro:IPR018375
InterPro:IPR036406

P

Seeded From UniProt

complete

involved_in

GO:0055114

oxidation-reduction process

GO_REF:0000002

ECO:0000256

match to sequence model evidence used in automatic assertion

InterPro:IPR001260
InterPro:IPR018375
InterPro:IPR036406

P

Seeded From UniProt

complete

enables

GO:0004109

coproporphyrinogen oxidase activity

GO_REF:0000003

ECO:0000501

evidence used in automatic assertion

EC:1.3.3.3

F

Seeded From UniProt

complete

involved_in

GO:0006783

heme biosynthetic process

PMID:8159699[3]
Reactome:R-HSA-189451

ECO:0000304

author statement supported by traceable reference used in manual assertion


P

Seeded From UniProt

complete

part_of

GO:0005739

mitochondrion

PMID:8407975[4]

ECO:0000304

author statement supported by traceable reference used in manual assertion

C

Seeded From UniProt

complete

enables

GO:0004109

coproporphyrinogen oxidase activity

PMID:9888388[5]

ECO:0000304

author statement supported by traceable reference used in manual assertion

F

Seeded From UniProt

complete

part_of

GO:0005758

mitochondrial intermembrane space

Reactome:R-HSA-189421

ECO:0000304

author statement supported by traceable reference used in manual assertion

C

Seeded From UniProt

complete

involved_in

GO:0055114

oxidation-reduction process

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0560

P

Seeded From UniProt

complete

involved_in

GO:0006779

porphyrin-containing compound biosynthetic process

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0627

P

Seeded From UniProt

complete

part_of

GO:0005739

mitochondrion

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0496

C

Seeded From UniProt

complete

involved_in

GO:0006783

heme biosynthetic process

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0350

P

Seeded From UniProt

complete

enables

GO:0016491

oxidoreductase activity

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0560

F

Seeded From UniProt

complete

part_of

GO:0005758

mitochondrial intermembrane space

GO_REF:0000039

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-SubCell:SL-0169

C

Seeded From UniProt

complete

involved_in

GO:0006782

protoporphyrinogen IX biosynthetic process

GO_REF:0000041

ECO:0000322

imported manually asserted information used in automatic assertion

UniPathway:UPA00251

P

Seeded From UniProt

complete

Notes

References

See Help:References for how to manage references in GONUTS.

  1. Lee, DS et al. (2005) Structural basis of hereditary coproporphyria. Proc. Natl. Acad. Sci. U.S.A. 102 14232-7 PubMed GONUTS page
  2. 2.0 2.1 2.2 2.3 Gaudet, P et al. (2011) Phylogenetic-based propagation of functional annotations within the Gene Ontology consortium. Brief. Bioinformatics 12 449-62 PubMed GONUTS page
  3. Martasek, P et al. (1994) Molecular cloning, sequencing, and functional expression of a cDNA encoding human coproporphyrinogen oxidase. Proc. Natl. Acad. Sci. U.S.A. 91 3024-8 PubMed GONUTS page
  4. Kohno, H et al. (1993) Coproporphyrinogen oxidase. Purification, molecular cloning, and induction of mRNA during erythroid differentiation. J. Biol. Chem. 268 21359-63 PubMed GONUTS page
  5. Rosipal, R et al. (1999) Systematic analysis of coproporphyrinogen oxidase gene defects in hereditary coproporphyria and mutation update. Hum. Mutat. 13 44-53 PubMed GONUTS page