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MT000144

UniProt Annotations

Entry Information

Gene Name	coproporphyrinogen oxidase
Protein Entry	HEM6_HUMAN
UniProt ID	P36551
Species	Human

Comments

Comment type	Description
Alternative Products	Event=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 Activity	Coproporphyrinogen-III + O(2) + 2 H(+) = protoporphyrinogen-IX + 2 CO(2) + 2 H(2)O.
Disease	Hereditary 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.
Function	Involved 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.
Pathway	Porphyrin-containing compound metabolism; protoporphyrin- IX biosynthesis; protoporphyrinogen-IX from coproporphyrinogen-III (O2 route): step 1/1.
Sequence Caution	Sequence=BAA04033.1; Type=Erroneous initiation; Note=Translation N-terminally extended.; Evidence={ECO:0000305};
Similarity	Belongs to the aerobic coproporphyrinogen-III oxidase family. {ECO:0000305}.
Subcellular Location	Mitochondrion intermembrane space.
Subunit	Homodimer. {ECO:0000269\|PubMed:16176984}.