Display Settings:

Format

Send to:

Choose Destination
We are sorry, but NCBI web applications do not support your browser and may not function properly. More information

CPOX coproporphyrinogen oxidase [ Homo sapiens (human) ]

Gene ID: 1371, updated on 6-Jul-2014
Official Symbol
CPOXprovided by HGNC
Official Full Name
coproporphyrinogen oxidaseprovided by HGNC
Primary source
HGNC:2321
See related
Ensembl:ENSG00000080819; HPRD:00417; MIM:612732; Vega:OTTHUMG00000160016
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Homo sapiens
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as
CPO; CPX; HCP
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]
See CPOX in Epigenomics, MapViewer
Location:
3q12
Exon count :
8
Annotation release Status Assembly Chr Location
106 current GRCh38 (GCF_000001405.26) 3 NC_000003.12 (98577836..98593624, complement)
105 previous assembly GRCh37.p13 (GCF_000001405.25) 3 NC_000003.11 (98296677..98312470, complement)

Chromosome 3 - NC_000003.12Genomic Context describing neighboring genes Neighboring gene ribosomal protein L38 pseudogene 4 Neighboring gene G protein-coupled receptor 15 Neighboring gene ribosomal protein L19 pseudogene 7 Neighboring gene WW domain containing E3 ubiquitin protein ligase 1 pseudogene 1

GeneRIFs: Gene References Into FunctionsWhat's a GeneRIF?

Products Interactant Other Gene Complex Source Pubs Description

Markers

Homology

Gene Ontology Provided by GOA

Function Evidence Code Pubs
coproporphyrinogen oxidase activity TAS
Traceable Author Statement
more info
 
protein homodimerization activity IDA
Inferred from Direct Assay
more info
PubMed 
structural constituent of eye lens IEA
Inferred from Electronic Annotation
more info
 
Process Evidence Code Pubs
heme biosynthetic process TAS
Traceable Author Statement
more info
 
oxidation-reduction process IEA
Inferred from Electronic Annotation
more info
 
porphyrin-containing compound metabolic process TAS
Traceable Author Statement
more info
 
protoporphyrinogen IX biosynthetic process IEA
Inferred from Electronic Annotation
more info
 
response to arsenic-containing substance IEA
Inferred from Electronic Annotation
more info
 
response to insecticide IEA
Inferred from Electronic Annotation
more info
 
response to iron ion IEA
Inferred from Electronic Annotation
more info
 
response to lead ion IEA
Inferred from Electronic Annotation
more info
 
response to methylmercury IEA
Inferred from Electronic Annotation
more info
 
small molecule metabolic process TAS
Traceable Author Statement
more info
 
Component Evidence Code Pubs
mitochondrial inner membrane IEA
Inferred from Electronic Annotation
more info
 
mitochondrial intermembrane space TAS
Traceable Author Statement
more info
 
mitochondrion IDA
Inferred from Direct Assay
more info
 
Preferred Names
oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
Names
oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
COX
coprogen oxidase
coproporphyrinogenase
coproporphyrinogen-III oxidase, mitochondrial
NP_000088.3

RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

Genomic

  1. NG_015994.1 

    Range
    5001..19166
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. NM_000097.5NP_000088.3  oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial precursor

    See proteins identical to NP_000088.3

    Status: REVIEWED

    Source sequence(s)
    AK290140, BC017210, BC023551
    Consensus CDS
    CCDS2932.1
    UniProtKB/Swiss-Prot
    P36551
    Related
    ENSP00000264193, OTTHUMP00000217368, ENST00000264193, OTTHUMT00000358900
    Conserved Domains (1) summary
    pfam01218
    Location:150453
    Blast Score: 1442
    Coprogen_oxidas; Coproporphyrinogen III oxidase

RefSeqs of Annotated Genomes: Homo sapiens Annotation Release 106

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCh38 Primary Assembly

Genomic

  1. NC_000003.12 

    Range
    98577836..98593624
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_005247125.2XP_005247182.1  

    Conserved Domains (1) summary
    pfam01218
    Location:150391
    Blast Score: 1120
    Coprogen_oxidas; Coproporphyrinogen III oxidase

Alternate HuRef

Genomic

  1. AC_000135.1 

    Range
    95670419..95684588
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

Alternate CHM1_1.1

Genomic

  1. NC_018914.2 

    Range
    98261466..98275631
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

Supplemental Content

Recent activity

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

See more...