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CPOX coproporphyrinogen oxidase [ Homo sapiens (human) ]

Gene ID: 1371, updated on 8-Dec-2018

Summary

Official Symbol
CPOXprovided by HGNC
Official Full Name
coproporphyrinogen oxidaseprovided by HGNC
Primary source
HGNC:HGNC:2321
See related
Ensembl:ENSG00000080819 MIM:612732
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
COX; 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]
Expression
Ubiquitous expression in bone marrow (RPKM 5.0), liver (RPKM 3.1) and 24 other tissues See more
Orthologs

Genomic context

See CPOX in Genome Data Viewer
Location:
3q11.2
Exon count:
10
Annotation release Status Assembly Chr Location
109 current GRCh38.p12 (GCF_000001405.38) 3 NC_000003.12 (98569837..98593684, 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

Genomic regions, transcripts, and products

Expression

  • Project title: HPA RNA-seq normal tissues
  • Description: RNA-seq was performed of tissue samples from 95 human individuals representing 27 different tissues in order to determine tissue-specificity of all protein-coding genes
  • BioProject: PRJEB4337
  • Publication: PMID 24309898
  • Analysis date: Wed Apr 4 07:08:55 2018

Bibliography

GeneRIFs: Gene References Into FunctionsWhat's a GeneRIF?

Pathways from BioSystems

  • Heme Biosynthesis, organism-specific biosystem (from WikiPathways)
    Heme Biosynthesis, organism-specific biosystemThe enzymatic process that produces heme is properly called porphyrin synthesis, as all the intermediates are tetrapyrroles that are chemically classified are porphyrins. The process is highly conser...
  • Heme biosynthesis, organism-specific biosystem (from REACTOME)
    Heme biosynthesis, organism-specific biosystemEight enzymes are involved in heme biosynthesis, four each in the mitochondria and the cytosol. The process starts in the mitochondria with the condensation of succinyl CoA (from the TCA cycle) and g...
  • Metabolic pathways, organism-specific biosystem (from KEGG)
    Metabolic pathways, organism-specific biosystem
    Metabolic pathways
  • Metabolism, organism-specific biosystem (from REACTOME)
    Metabolism, organism-specific biosystemMetabolic processes in human cells generate energy through the oxidation of molecules consumed in the diet and mediate the synthesis of diverse essential molecules not taken in the diet as well as th...
  • Metabolism of porphyrins, organism-specific biosystem (from REACTOME)
    Metabolism of porphyrins, organism-specific biosystemPorphyrins are heterocyclic macrocycles, consisting of four pyrrole subunits (tetrapyrrole) linked by four methine (=CH-) bridges. The extensive conjugated porphyrin macrocycle is chromatic and the n...
  • Porphyrin and chlorophyll metabolism, organism-specific biosystem (from KEGG)
    Porphyrin and chlorophyll metabolism, organism-specific biosystem
    Porphyrin and chlorophyll metabolism
  • Porphyrin and chlorophyll metabolism, conserved biosystem (from KEGG)
    Porphyrin and chlorophyll metabolism, conserved biosystem
    Porphyrin and chlorophyll metabolism
  • heme biosynthesis, organism-specific biosystem (from BIOCYC)
    heme biosynthesis, organism-specific biosystemGeneral Background Heme (protoheme, heme b) is an iron-containing porphyrin, that belongs to the family of macrocyclic tetrapyrroles. It has a diverse range of biological functions as a prosthetic g...
  • heme biosynthesis I (aerobic), conserved biosystem (from BIOCYC)
    heme biosynthesis I (aerobic), conserved biosystemGeneral Background |FRAME: Hemes Heme| (protoheme, heme b) is an iron-containing prosthetic group found in many essential proteins including cytochromes and heme-containing globins. In addition to i...
  • heme biosynthesis from uroporphyrinogen-III I, organism-specific biosystem (from BIOCYC)
    heme biosynthesis from uroporphyrinogen-III I, organism-specific biosystemGeneral Background : Hemes Heme (protoheme, heme b) is an iron-containing prosthetic group found in many essential proteins including cytochromes and heme-containing globins. In addition to its role...
  • superpathway of heme biosynthesis from glycine, conserved biosystem (from BIOCYC)
    superpathway of heme biosynthesis from glycine, conserved biosystemGeneral Background Heme (protoheme, heme b) is an iron-containing prosthetic group found in many essential proteins including cytochromes and heme-containing globins. In addition to its role in oxi...

Interactions

Products Interactant Other Gene Complex Source Pubs Description

General gene information

Markers

Homology

Gene Ontology Provided by GOA

Function Evidence Code Pubs
coproporphyrinogen oxidase activity IBA
Inferred from Biological aspect of Ancestor
more info
PubMed 
coproporphyrinogen oxidase activity TAS
Traceable Author Statement
more info
 
protein homodimerization activity IDA
Inferred from Direct Assay
more info
PubMed 
Process Evidence Code Pubs
heme biosynthetic process TAS
Traceable Author Statement
more info
 
oxidation-reduction process IEA
Inferred from Electronic Annotation
more info
 
protoporphyrinogen IX biosynthetic process from glutamate IBA
Inferred from Biological aspect of Ancestor
more info
PubMed 
Component Evidence Code Pubs
cytoplasm IBA
Inferred from Biological aspect of Ancestor
more info
PubMed 
cytosol IBA
Inferred from Biological aspect of Ancestor
more info
PubMed 
cytosol IDA
Inferred from Direct Assay
more info
 
mitochondrial intermembrane space TAS
Traceable Author Statement
more info
 
mitochondrion IDA
Inferred from Direct Assay
more info
 

General protein information

Preferred Names
oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
Names
coprogen oxidase
coproporphyrinogenase
NP_000088.3
XP_005247182.1

NCBI Reference Sequences (RefSeq)

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_015994.2 RefSeqGene

    Range
    5001..19166
    Download
    GenBank, FASTA, Sequence Viewer (Graphics), LRG_1077

mRNA and Protein(s)

  1. NM_000097.7NP_000088.3  oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial precursor

    See identical proteins and their annotated locations for NP_000088.3

    Status: REVIEWED

    Source sequence(s)
    AK290140, BC017210, BC023551
    Consensus CDS
    CCDS2932.1
    UniProtKB/Swiss-Prot
    P36551
    Related
    ENSP00000497326.1, ENST00000647941.1
    Conserved Domains (1) summary
    pfam01218
    Location:151453
    Coprogen_oxidas; Coproporphyrinogen III oxidase

RefSeqs of Annotated Genomes: Homo sapiens Annotation Release 109

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

Reference GRCh38.p12 Primary Assembly

Genomic

  1. NC_000003.12 Reference GRCh38.p12 Primary Assembly

    Range
    98569837..98593684 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_005247125.4XP_005247182.1  oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial isoform X1

    Conserved Domains (1) summary
    pfam01218
    Location:151391
    Coprogen_oxidas; Coproporphyrinogen III oxidase

RNA

  1. XR_001740025.2 RNA Sequence

  2. XR_001740028.1 RNA Sequence

  3. XR_001740027.1 RNA Sequence

  4. XR_001740026.1 RNA Sequence

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