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MGP000670

Record overview

MGPD IDMGP000670
Gene ID1371
SpeciesHomo sapiens (Human)
Gene Namecoproporphyrinogen oxidase
Gene Symbol CPOX
SynonymsCPO; CPX; HCP;
Alternate namesoxygen-dependent coproporphyrinogen-III oxidase, mitochondrial; COX; coprogen oxidase; coproporphyrinogen-III oxidase, mitochondrial; coproporphyrinogenase;
Chromosome3
Map Location3q12
EC Number1.3.3.3
SummaryThe protein encoded by this gene is the sixth enzyme of the heme biosynthetic pathway. The encoded enzyme is soluble and found in the intermembrane space of mitochondria. This enzyme catalyzes the stepwise oxidative decarboxylation of coproporphyrinogen III to protoporphyrinogen IX, a precursor of heme. Defects in this gene are a cause of hereditary coproporphyria (HCP).[provided by RefSeq, Oct 2009]
OrthologsView orthologs and multiple alignments for CPOX

Proteins

oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial precursor
Refseq ID:NP_000088
Protein GI:41393599
UniProt ID:P36551
mRNA ID:NM_000097
Length:454
RefSeq Status:
MALQLGRLSSGPCWLVARGGCGGPRAWSQCGGGGLRAWSQRSAAGRVCRPPGPAGTEQSRGLGHGSTSRGGPWVGTGLAAALAGLVGLATAAFGHVQRAE
MLPKTSGTRATSLGRPEEEEDELAHRCSSFMAPPVTDLGELRRRPGDMKTKMELLILETQAQVCQALAQVDGGANFSVDRWERKEGGGGISCVLQDGCVF
EKAGVSISVVHGNLSEEAAKQMRSRGKVLKTKDGKLPFCAMGVSSVIHPKNPHAPTIHFNYRYFEVEEADGNKQWWFGGGCDLTPTYLNQEDAVHFHRTL
KEACDQHGPDLYPKFKKWCDDYFFIAHRGERRGIGGIFFDDLDSPSKEEVFRFVQSCARAVVPSYIPLVKKHCDDSFTPQEKLWQQLRRGRYVEFNLLYD
RGTKFGLFTPGSRIESILMSLPLTARWEYMHSPSENSKEAEILEVLRHPRDWVR
 
transit_peptide: 1..110
calculated_mol_wt: 10922
peptide sequence: 
MALQLGRLSSGPCWLVARGGCGGPRAWSQCGGGGLRAWSQRSAAGRVCRPPGPAGTEQSRGLGHGSTSRGGPWVGTGLAAALAGLVGLATAAFGHVQRAE
MLPKTSGTRA

mat_peptide: 111..454
product: oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
calculated_mol_wt: 39249
peptide sequence: 
TSLGRPEEEEDELAHRCSSFMAPPVTDLGELRRRPGDMKTKMELLILETQAQVCQALAQVDGGANFSVDRWERKEGGGGISCVLQDGCVFEKAGVSISVV
HGNLSEEAAKQMRSRGKVLKTKDGKLPFCAMGVSSVIHPKNPHAPTIHFNYRYFEVEEADGNKQWWFGGGCDLTPTYLNQEDAVHFHRTLKEACDQHGPD
LYPKFKKWCDDYFFIAHRGERRGIGGIFFDDLDSPSKEEVFRFVQSCARAVVPSYIPLVKKHCDDSFTPQEKLWQQLRRGRYVEFNLLYDRGTKFGLFTP
GSRIESILMSLPLTARWEYMHSPSENSKEAEILEVLRHPRDWVR
 
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