Format

Send to:

Choose Destination
    • Showing Current items.

    CPOX coproporphyrinogen oxidase [ Homo sapiens (human) ]

    Gene ID: 1371, updated on 8-Jul-2021

    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; HARPO
    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
    NEW
    Try the new Gene table
    Try the new Transcript table

    Genomic context

    See CPOX in Genome Data Viewer
    Location:
    3q11.2
    Exon count:
    10
    Annotation release Status Assembly Chr Location
    109.20210514 current GRCh38.p13 (GCF_000001405.39) 3 NC_000003.12 (98569837..98593684, complement)
    105.20201022 previous assembly GRCh37.p13 (GCF_000001405.25) 3 NC_000003.11 (98298290..98312455, complement)

    Chromosome 3 - NC_000003.12Genomic Context describing neighboring genes Neighboring gene RPL38 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 Functions

    What's a GeneRIF?

    Pathways from PubChem

    Interactions

    Products Interactant Other Gene Complex Source Pubs Description

    General gene information

    Markers

    Homology

    Gene Ontology Provided by GOA

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

    General protein information

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

    NCBI Reference Sequences (RefSeq)

    NEW Try the new Transcript table

    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, ENST00000647941.2
      Conserved Domains (1) summary
      pfam01218
      Location:151453
      Coprogen_oxidas; Coproporphyrinogen III oxidase

    RefSeqs of Annotated Genomes: Homo sapiens Updated Annotation Release 109.20210514

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

    Reference GRCh38.p13 Primary Assembly

    Genomic

    1. NC_000003.12 Reference GRCh38.p13 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

    Support Center