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MGP000670

UniProt Annotations

Entry Information
Gene Namecoproporphyrinogen oxidase
Protein EntryHEM6_HUMAN
UniProt IDP36551
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=P36551-1; Sequence=Displayed; Name=2; IsoId=P36551-2; Sequence=VSP_057182, VSP_057183; Note=No experimental confirmation available.;
Catalytic ActivityCoproporphyrinogen-III + O(2) + 2 H(+) = protoporphyrinogen-IX + 2 CO(2) + 2 H(2)O.
DiseaseHereditary coproporphyria (HCP) [MIM:121300]: A form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. Hereditary coproporphyria is an acute hepatic porphyria characterized by skin photosensitivity, attacks of abdominal pain, neurological disturbances, and psychiatric symptoms. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. Hereditary coproporphyria is biochemically characterized by overexcretion of coproporphyrin III in the urine and in the feces. {ECO:0000269|PubMed:12181641, ECO:0000269|PubMed:15896662, ECO:0000269|PubMed:16398658, ECO:0000269|PubMed:7757079, ECO:0000269|PubMed:7849704, ECO:0000269|PubMed:8012360, ECO:0000269|PubMed:8990017, ECO:0000269|PubMed:9048920, ECO:0000269|PubMed:9298818, ECO:0000269|PubMed:9888388}. Note=The disease is caused by mutations affecting the gene represented in this entry.
FunctionInvolved in the heme biosynthesis. Catalyzes the aerobic oxidative decarboxylation of propionate groups of rings A and B of coproporphyrinogen-III to yield the vinyl groups in protoporphyrinogen-IX.
PathwayPorphyrin-containing compound metabolism; protoporphyrin- IX biosynthesis; protoporphyrinogen-IX from coproporphyrinogen-III (O2 route): step 1/1.
Sequence CautionSequence=BAA04033.1; Type=Erroneous initiation; Note=Translation N-terminally extended.; Evidence={ECO:0000305};
SimilarityBelongs to the aerobic coproporphyrinogen-III oxidase family. {ECO:0000305}.
Subcellular LocationMitochondrion intermembrane space.
SubunitHomodimer. {ECO:0000269|PubMed:16176984}.
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