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    CPOX coproporphyrinogen oxidase [ Homo sapiens (human) ]

    Gene ID: 1371, updated on 8-Oct-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; 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
    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 Jun 15 11:32:44 2016

    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, OTTHUMP00000217368, ENST00000647941.1
      Conserved Domains (1) summary
      pfam01218
      Location:151453
      Coprogen_oxidas; Coproporphyrinogen III oxidase

    RefSeqs of Annotated Genomes: Homo sapiens Annotation Release 109 details...Open this link in a new tab

    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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