CPOX
entrez
1371
canonical
uniprot
P36551
canonical
ensembl
ENSG00000080819
resolver
ensp
ENSP00000497326
resolver
ensp
ENSP00000652855
resolver
ensp
ENSP00000652858
resolver
ensp
ENSP00000781266
resolver
genesymbol
CPOX
resolver
genesymbol_syn
CPO
resolver
genesymbol_syn
CPX
resolver
hgnc
HGNC:2321
resolver
uniprot
A8K275
resolver
uniprot
B4DSD5
resolver
uniprot
Q14060
resolver
uniprot
Q53F08
resolver
uniprot
Q8IZ45
resolver
uniprot
Q96AF3
resolver
uniprot_entry
HEM6_HUMAN
resolver
uniprot_sec
A8K275
resolver
uniprot_sec
B4DSD5
resolver
uniprot_sec
Q14060
resolver
uniprot_sec
Q53F08
resolver
uniprot_sec
Q8IZ45
resolver
uniprot_sec
Q96AF3
resolver
amino_acid_sequence
MALQLGRLSSGPCWLVARGGCGGPRAWSQCGGGGLRAWSQRSAAGRVCRPPGPAGTEQSRGLGHGSTSRGGPWVGTGLAAALAGLVGLATAAFGHVQRAEMLPKTSGTRATSLGRPEEEEDELAHRCSSFMAPPVTDLGELRRRPGDMKTKMELLILETQAQVCQALAQVDGGANFSVDRWERKEGGGGISCVLQDGCVFEKAGVSISVVHGNLSEEAAKQMRSRGKVLKTKDGKLPFCAMGVSSVIHPKNPHAPTIHFNYRYFEVEEADGNKQWWFGGGCDLTPTYLNQEDAVHFHRTLKEACDQHGPDLYPKFKKWCDDYFFIAHRGERRGIGGIFFDDLDSPSKEEVFRFVQSCARAVVPSYIPLVKKHCDDSFTPQEKLWQQLRRGRYVEFNLLYDRGTKFGLFTPGSRIESILMSLPLTARWEYMHSPSENSKEAEILEVLRHPRDWVR
uniprot
proteins
disease_involvement
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:7849704, ECO:0000269|PubMed:8012360, ECO:0000269|PubMed:8990017, ECO:0000269|PubMed:9048920, ECO:0000269|PubMed:9298818, ECO:0000269|PubMed:9843038, ECO:0000269|PubMed:9888388}. Note=The disease is caused by variants affecting the gene represented in this entry.; DISEASE: Harderoporphyria (HARPO) [MIM:618892]: An autosomal recessive form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. HARPO is a rare erythropoietic variant form characterized by neonatal hemolytic anemia, sometimes accompanied by skin lesions, and massive excretion of harderoporphyrin in feces. {ECO:0000269|PubMed:21103937, ECO:0000269|PubMed:7757079}. Note=The disease is caused by variants affecting the gene represented in this entry.
uniprot
proteins
ec_number
1.3.3.3
uniprot
proteins
function
FUNCTION: Catalyzes the aerobic oxidative decarboxylation of propionate groups of rings A and B of coproporphyrinogen-III to yield the vinyl groups in protoporphyrinogen-IX and participates to the sixth step in the heme biosynthetic pathway. {ECO:0000269|PubMed:8159699}.
uniprot
proteins
mass_dalton
50152
uniprot
proteins
mutagenesis
MUTAGEN 392..418; /note="Missing: Loss for dimerization."; /evidence="ECO:0000269|PubMed:16176984
uniprot
proteins
pathway_participation
PATHWAY: Porphyrin-containing compound metabolism; protoporphyrin-IX biosynthesis; protoporphyrinogen-IX from coproporphyrinogen-III (O2 route): step 1/1. {ECO:0000269|PubMed:8159699}.
uniprot
proteins
protein_family
Aerobic coproporphyrinogen-III oxidase family
uniprot
proteins
pubmed
12181641
uniprot
proteins
pubmed
12862310
uniprot
proteins
pubmed
14702039
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
15896662
uniprot
proteins
pubmed
16176984
uniprot
proteins
pubmed
16398658
uniprot
proteins
pubmed
21103937
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
23186163
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
25944712
uniprot
proteins
pubmed
7757079
uniprot
proteins
pubmed
7849704
uniprot
proteins
pubmed
7987309
uniprot
proteins
pubmed
8012360
uniprot
proteins
pubmed
8159699
uniprot
proteins
pubmed
8286403
uniprot
proteins
pubmed
8990017
uniprot
proteins
pubmed
9048920
uniprot
proteins
pubmed
9298818
uniprot
proteins
pubmed
9843038
uniprot
proteins
pubmed
9888388
uniprot
proteins
sequence_length
454
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion intermembrane space {ECO:0000250|UniProtKB:Q3B7D0}.
uniprot
proteins