MitoProteome Database

MT000144

Record overview

MITO ID	MT000144
Gene ID	1371
Species	Homo sapiens (Human)
Gene Name	coproporphyrinogen oxidase
Gene Symbol	CPOX
Synonyms	CPO; CPX; HCP;
Alternate names	oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial; COX; coprogen oxidase; coproporphyrinogenase; coproporphyrinogen-III oxidase, mitochondrial;
Chromosome	3
Map Location	3q12
EC Number	1.3.3.3
Summary	The 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]
Orthologs	View 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

N
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