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Aliases for CPOX Gene

Aliases for CPOX Gene

  • Coproporphyrinogen Oxidase 2 3 5
  • Coproporphyrinogenase 3 4
  • Coprogen Oxidase 3 4
  • EC 4 63
  • COX 3 4
  • CPO 3 4
  • CPX 3 4
  • Coproporphyrinogen Oxidase (Coproporphyria, Harderoporphyria) 2
  • Coproporphyrinogen-III Oxidase, Mitochondrial 3
  • Coproporphyria 2
  • HCP 3

External Ids for CPOX Gene

Previous HGNC Symbols for CPOX Gene

  • CPO

Previous GeneCards Identifiers for CPOX Gene

  • GC03M099619
  • GC03M099780
  • GC03M098298
  • GC03M095670
  • GC03M098239

Summaries for CPOX Gene

Entrez Gene Summary for CPOX Gene

  • 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]

GeneCards Summary for CPOX Gene

CPOX (Coproporphyrinogen Oxidase) is a Protein Coding gene. Diseases associated with CPOX include Coproporphyria and Porphyria Cutanea Tarda. Among its related pathways are Metabolism and Porphyrin and chlorophyll metabolism. GO annotations related to this gene include protein homodimerization activity and structural constituent of eye lens.

UniProtKB/Swiss-Prot for CPOX Gene

  • Involved in the heme biosynthesis. Catalyzes the aerobic oxidative decarboxylation of propionate groups of rings A and B of coproporphyrinogen-III to yield the vinyl groups in protoporphyrinogen-IX.

Gene Wiki entry for CPOX Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for CPOX Gene

Genomics for CPOX Gene

Regulatory Elements for CPOX Gene

Enhancers for CPOX Gene
GeneHancer Identifier Score Enhancer Sources TSS distance (kb) Number of Genes Away Size (kb) Transcription Factor Binding Sites within enhancer Other Gene Targets for Enhancer

Enhancers around CPOX on UCSC Golden Path with GeneCards custom track

Promoters for CPOX Gene
Ensembl Regulatory Elements (ENSRs) TSS Distance (bp) Size (bp) Binding Sites for Transcription Factors within promoters

ENSRs around CPOX on UCSC Golden Path with GeneCards custom track

Genomic Location for CPOX Gene

98,521,132 bp from pter
98,593,723 bp from pter
72,592 bases
Minus strand

Genomic View for CPOX Gene

Genes around CPOX on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
CPOX Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for CPOX Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for CPOX Gene

Proteins for CPOX Gene

  • Protein details for CPOX Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
    Protein Accession:
    Secondary Accessions:
    • A8K275
    • B4DSD5
    • Q14060
    • Q53F08
    • Q8IZ45
    • Q96AF3

    Protein attributes for CPOX Gene

    454 amino acids
    Molecular mass:
    50152 Da
    Quaternary structure:
    • Homodimer.
    • Sequence=BAA04033.1; Type=Erroneous initiation; Note=Translation N-terminally extended.; Evidence={ECO:0000305};

    Three dimensional structures from OCA and Proteopedia for CPOX Gene

    Alternative splice isoforms for CPOX Gene


neXtProt entry for CPOX Gene

Proteomics data for CPOX Gene at MOPED

Selected DME Specific Peptides for CPOX Gene

Post-translational modifications for CPOX Gene

  • Ubiquitination at Lys 184
  • Modification sites at PhosphoSitePlus

Other Protein References for CPOX Gene

ENSEMBL proteins:
REFSEQ proteins:

Domains & Families for CPOX Gene

Protein Domains for CPOX Gene

Suggested Antigen Peptide Sequences for CPOX Gene

GenScript: Design optimal peptide antigens:

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the aerobic coproporphyrinogen-III oxidase family.
  • Belongs to the aerobic coproporphyrinogen-III oxidase family.
genes like me logo Genes that share domains with CPOX: view

No data available for Gene Families for CPOX Gene

Function for CPOX Gene

Molecular function for CPOX Gene

UniProtKB/Swiss-Prot CatalyticActivity:
Coproporphyrinogen-III + O(2) + 2 H(+) = protoporphyrinogen-IX + 2 CO(2) + 2 H(2)O.
UniProtKB/Swiss-Prot Function:
Involved in the heme biosynthesis. Catalyzes the aerobic oxidative decarboxylation of propionate groups of rings A and B of coproporphyrinogen-III to yield the vinyl groups in protoporphyrinogen-IX.

Enzyme Numbers (IUBMB) for CPOX Gene

genes like me logo Genes that share phenotypes with CPOX: view

Human Phenotype Ontology for CPOX Gene

HPO Id HPO Name Alternative Ids Definition Synonyms

Animal Model Products

No data available for Gene Ontology (GO) - Molecular Function , Animal Models , Transcription Factor Targets and HOMER Transcription for CPOX Gene

Localization for CPOX Gene

Subcellular locations from UniProtKB/Swiss-Prot for CPOX Gene

Mitochondrion intermembrane space.

Subcellular locations from

Jensen Localization Image for CPOX Gene COMPARTMENTS Subcellular localization image for CPOX gene
Compartment Confidence
mitochondrion 5
cytosol 3
chloroplast 1
nucleus 1
plasma membrane 1

Gene Ontology (GO) - Cellular Components for CPOX Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IEA,IDA --
GO:0016021 integral component of membrane IEA --
genes like me logo Genes that share ontologies with CPOX: view

Pathways & Interactions for CPOX Gene

genes like me logo Genes that share pathways with CPOX: view

Pathways by source for CPOX Gene

UniProtKB/Swiss-Prot P36551-HEM6_HUMAN

  • Pathway: Porphyrin-containing compound metabolism; protoporphyrin-IX biosynthesis; protoporphyrinogen-IX from coproporphyrinogen-III (O2 route): step 1/1.

Gene Ontology (GO) - Biological Process for CPOX Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006778 porphyrin-containing compound metabolic process TAS --
GO:0006782 protoporphyrinogen IX biosynthetic process IEA --
GO:0006783 heme biosynthetic process IEA,TAS --
GO:0010039 response to iron ion IEA --
genes like me logo Genes that share ontologies with CPOX: view

No data available for SIGNOR curated interactions for CPOX Gene

Drugs & Compounds for CPOX Gene

(30) Drugs for CPOX Gene - From: HMDB and Novoseek

Name Status Disease Links Group Role Mechanism of Action Clinical Trials
Protoporphyrin IX Experimental Pharma 6
Coproporphyrin Iii Experimental Pharma 0
heme Pharma Agonist 0

(42) Additional Compounds for CPOX Gene - From: Novoseek and HMDB

Name Synonyms Role CAS Number PubChem IDs PubMed IDs
  • Dioxygen
  • Molecular oxygen
  • O2
  • Oxygen
  • Oxygen molecule
carbon dioxide
  • Carbon oxide
  • Carbon-12 dioxide
  • Carbonic acid anhydride
  • Carbonic acid gas
  • Carbonic anhydride
Coproporphyrinogen III
  • 3,8,13,17-Tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropanoate
  • 3,8,13,17-Tetramethyl-5,10,15,20,22,24-hexahydroporphyrin-2,7,12,18-tetrapropanoic acid
  • 5,10,15,20,22,24-Hexahydro-3,8,13,17-tetramethyl-2,7,12,18-Porphinetetrapropionate
  • 5,10,15,20,22,24-Hexahydro-3,8,13,17-tetramethyl-2,7,12,18-Porphinetetrapropionic acid
  • 5,10,15,20,22,24-Hexahydro-3,8,13,17-tetramethyl-21H,23H-Porphine-2,7,12,18-tetrapropanoate
  • Formyl coenzyme A
Hydrogen Ion
  • Hydrogen cation
  • Hydron
  • Proton
genes like me logo Genes that share compounds with CPOX: view

Transcripts for CPOX Gene

Unigene Clusters for CPOX Gene

Coproporphyrinogen oxidase:
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for CPOX Gene

ExUns: 1a · 1b · 1c · 1d ^ 2a · 2b · 2c ^ 3a · 3b ^ 4a · 4b ^ 5 ^ 6a · 6b ^ 7
SP1: -
SP2: - -
SP4: - -

Relevant External Links for CPOX Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for CPOX Gene

mRNA expression in normal human tissues for CPOX Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

Protein differential expression in normal tissues from HIPED for CPOX Gene

This gene is overexpressed in Bone (13.7), Peripheral blood mononuclear cells (10.0), and Fetal Liver (9.5).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, PaxDb, MOPED, and MaxQB for CPOX Gene

SOURCE GeneReport for Unigene cluster for CPOX Gene Hs.476982

genes like me logo Genes that share expression patterns with CPOX: view

Protein tissue co-expression partners for CPOX Gene

- Elite partner

Primer Products

No data available for mRNA differential expression in normal tissues and mRNA Expression by UniProt/SwissProt for CPOX Gene

Orthologs for CPOX Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for CPOX Gene

Organism Taxonomy Gene Similarity Type Details
(Bos Taurus)
Mammalia CPOX 35
  • 87.2 (n)
  • 86.61 (a)
  • 86 (a)
(Mus musculus)
Mammalia Cpox 35
  • 83.41 (n)
  • 81.9 (a)
Cpox 16
Cpox 36
  • 81 (a)
(Pan troglodytes)
Mammalia CPOX 35
  • 99.49 (n)
  • 98.68 (a)
  • 99 (a)
(Rattus norvegicus)
Mammalia Cpox 35
  • 83.94 (n)
  • 82.13 (a)
(Canis familiaris)
Mammalia CPOX 36
  • 94 (a)
(Monodelphis domestica)
Mammalia CPOX 36
  • 74 (a)
(Ornithorhynchus anatinus)
Mammalia CPOX 36
  • 58 (a)
(Gallus gallus)
Aves CPOX 35
  • 73.67 (n)
  • 76.33 (a)
  • 70 (a)
(Anolis carolinensis)
Reptilia CPOX 36
  • 85 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia cpox 35
  • 69.57 (n)
  • 73.35 (a)
(Danio rerio)
Actinopterygii -- 35
cpox 35
  • 66.76 (n)
  • 71.08 (a)
cpox 36
  • 60 (a)
rainbow trout
(Oncorhynchus mykiss)
Actinopterygii Omy.10575 35
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP004749 35
  • 61.21 (n)
  • 64.81 (a)
fruit fly
(Drosophila melanogaster)
Insecta Coprox 35
  • 60.2 (n)
  • 63.16 (a)
Coprox 36
  • 59 (a)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_AGR030C 35
  • 55.08 (n)
  • 57.7 (a)
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0F18546g 35
  • 54.21 (n)
  • 54.43 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes HEM13 35
  • 54.97 (n)
  • 55.41 (a)
HEM13 36
  • 53 (a)
HEM13 38
thale cress
(Arabidopsis thaliana)
eudicotyledons LIN2 35
  • 52.7 (n)
  • 51.89 (a)
Alicante grape
(Vitis vinifera)
eudicotyledons Vvi.10363 35
(Hordeum vulgare)
Liliopsida Hv.138 35
(Oryza sativa)
Liliopsida Os.17919 35
Os04g0610800 35
  • 60.7 (n)
  • 58.78 (a)
(Triticum aestivum)
Liliopsida Ta.2884 35
sea squirt
(Ciona intestinalis)
Ascidiacea Cin.1210 35
bread mold
(Neurospora crassa)
Ascomycetes NCU01546 35
  • 54.85 (n)
  • 54.29 (a)
fission yeast
(Schizosaccharomyces pombe)
Schizosaccharomycetes hem13 35
  • 56 (n)
  • 52.33 (a)
sea squirt
(Ciona savignyi)
Ascidiacea -- 36
  • 58 (a)
Species with no ortholog for CPOX:
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • beta proteobacteria (Neisseria meningitidis)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • worm (Caenorhabditis elegans)

Evolution for CPOX Gene

Gene Tree for CPOX (if available)
Gene Tree for CPOX (if available)

Paralogs for CPOX Gene

No data available for Paralogs for CPOX Gene

Variants for CPOX Gene

Sequence variations from dbSNP and Humsavar for CPOX Gene

SNP ID Clin Chr 03 pos Sequence Context AA Info Type
VAR_002152 Hereditary coproporphyria (HCP)
VAR_002153 Hereditary coproporphyria (HCP)
VAR_002154 Hereditary coproporphyria (HCP)
VAR_002155 Hereditary coproporphyria (HCP)
rs1131857 - 98,588,852(-) TAGGC(A/C)ACAAG reference, missense

Structural Variations from Database of Genomic Variants (DGV) for CPOX Gene

Variant ID Type Subtype PubMed ID
dgv293n21 CNV Loss 19592680
nsv834775 CNV Gain 17160897

Variation tolerance for CPOX Gene

Residual Variation Intolerance Score: 67.7% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 11.67; 93.20% of all genes are more intolerant (likely to be disease-causing)

Relevant External Links for CPOX Gene

Human Gene Mutation Database (HGMD)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for CPOX Gene

Disorders for CPOX Gene

MalaCards: The human disease database

(16) MalaCards diseases for CPOX Gene - From: OMIM, GeneTests, Orphanet, Swiss-Prot, DISEASES, Novoseek, and GeneCards

Disorder Aliases PubMed IDs
  • harderoporphyria
porphyria cutanea tarda
  • porphyria, hepatoerythropoietic
  • disorder of porphyrin and hem metabolism
protoporphyria, erythropoietic, autosomal recessive
  • erythropoietic protoporphyria
porphyria variegata
  • variegate porphyria
- elite association - COSMIC cancer census association via MalaCards
Search CPOX in MalaCards View complete list of genes associated with diseases


  • Hereditary coproporphyria (HCP) [MIM:121300]: A form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. Hereditary coproporphyria is an acute hepatic porphyria characterized by skin photosensitivity, attacks of abdominal pain, neurological disturbances, and psychiatric symptoms. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. Hereditary coproporphyria is biochemically characterized by overexcretion of coproporphyrin III in the urine and in the feces. {ECO:0000269 PubMed:12181641, ECO:0000269 PubMed:15896662, ECO:0000269 PubMed:16398658, ECO:0000269 PubMed:7757079, ECO:0000269 PubMed:7849704, ECO:0000269 PubMed:8012360, ECO:0000269 PubMed:8990017, ECO:0000269 PubMed:9048920, ECO:0000269 PubMed:9298818, ECO:0000269 PubMed:9888388}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Relevant External Links for CPOX

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
Atlas of Genetics and Cytogenetics in Oncology and Haematology:
genes like me logo Genes that share disorders with CPOX: view

No data available for Genatlas for CPOX Gene

Publications for CPOX Gene

  1. A molecular defect in coproporphyrinogen oxidase gene causing harderoporphyria, a variant form of hereditary coproporphyria. (PMID: 7757079) Lamoril J. … Nordmann Y. (Hum. Mol. Genet. 1995) 2 3 4 67
  2. Cloning, expression, and biochemical properties of CPOX4, a genetic variant of coproporphyrinogen oxidase that affects susceptibility to mercury toxicity in humans. (PMID: 19339664) Li T. … Woods J.S. (Toxicol. Sci. 2009) 3 23
  3. Investigation of the catalytic and structural roles of conserved histidines of human coproporphyrinogen oxidase using site-directed mutagenesis. (PMID: 17179900) Gitter S.J. … Jones M.A. (Med. Sci. Monit. 2007) 3 23
  4. The association between a genetic polymorphism of coproporphyrinogen oxidase, dental mercury exposure and neurobehavioral response in humans. (PMID: 16343843) Echeverria D. … Garabedian C.E. (Neurotoxicol Teratol 2006) 3 23
  5. Mutations in human CPO gene predict clinical expression of either hepatic hereditary coproporphyria or erythropoietic harderoporphyria. (PMID: 16159891) Schmitt C. … Puy H. (Hum. Mol. Genet. 2005) 3 23

Products for CPOX Gene

Sources for CPOX Gene