Coproporphyrinogen III oxidase

From Wikipedia, the free encyclopedia
Jump to: navigation, search
Coproporphyrinogen oxidase
Protein CPOX PDB 2aex.png
PDB rendering based on 2aex.
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols CPOX ; CPO; CPX; HCP
External IDs OMIM612732 MGI104841 HomoloGene76 ChEMBL: 1681618 GeneCards: CPOX Gene
EC number
RNA expression pattern
PBB GE CPOX 204172 at tn.png
More reference expression data
Species Human Mouse
Entrez 1371 12892
Ensembl ENSG00000080819 ENSMUSG00000022742
UniProt P36551 P36552
RefSeq (mRNA) NM_000097 NM_007757
RefSeq (protein) NP_000088 NP_031783
Location (UCSC) Chr 3:
98.24 – 98.31 Mb
Chr 16:
58.67 – 58.68 Mb
PubMed search [1] [2]
PDB 1vju EBI.jpg
coproporphyrinogen iii oxidase from leishmania major
Symbol Coprogen_oxidas
Pfam PF01218
InterPro IPR001260

Coproporphyrinogen-III oxidase, mitochondrial is an enzyme that in humans is encoded by the CPOX gene.[1][2][3] A genetic defect in the enzyme results in a reduced production of heme in animals. The medical condition associated with this enzyme defect is called hereditary coproporphyria.[4] [5]

It is an enzyme involved in the sixth step of porphyrin metabolism it catalyses the oxidative decarboxylation of coproporphyrinogen III to proto-porphyrinogen IX in the haem and chlorophyll biosynthetic pathways.[2][6] The protein is a homodimer containing two internally bound iron atoms per molecule of native protein.[7] The enzyme is active in the presence of molecular oxygen that acts as an electron acceptor. The enzyme is widely distributed having been found in a variety of eukaryotic and prokaryotic sources.


  1. ^ Lamoril J, Martasek P, Deybach JC, Da Silva V, Grandchamp B, Nordmann Y (Feb 1995). "A molecular defect in coproporphyrinogen oxidase gene causing harderoporphyria, a variant form of hereditary coproporphyria". Human Molecular Genetics 4 (2): 275–8. doi:10.1093/hmg/4.2.275. PMID 7757079. 
  2. ^ a b Kohno H, Furukawa T, Yoshinaga T, Tokunaga R, Taketani S (Oct 1993). "Coproporphyrinogen oxidase. Purification, molecular cloning, and induction of mRNA during erythroid differentiation". The Journal of Biological Chemistry 268 (28): 21359–63. PMID 8407975. 
  3. ^ "Entrez Gene: CPOX coproporphyrinogen oxidase". 
  4. ^ "Hereditary coproporphyria". Genetic and Rare Diseases Information Center. National Institutes of Health. Retrieved 8 August 2011. 
  5. ^ "CPOX". Genetics Home Reference. Retrieved 8 August 2011. 
  6. ^ Madsen O, Sandal L, Sandal NN, Marcker KA (Oct 1993). "A soybean coproporphyrinogen oxidase gene is highly expressed in root nodules". Plant Molecular Biology 23 (1): 35–43. doi:10.1007/BF00021417. PMID 8219054. 
  7. ^ Camadro JM, Chambon H, Jolles J, Labbe P (May 1986). "Purification and properties of coproporphyrinogen oxidase from the yeast Saccharomyces cerevisiae". European Journal of Biochemistry / FEBS 156 (3): 579–87. doi:10.1111/j.1432-1033.1986.tb09617.x. PMID 3516695. 

Further reading[edit]

Heme synthesis—note that some reactions occur in the cytoplasm and some in the mitochondrion (yellow)

External links[edit]

This article incorporates text from the public domain Pfam and InterPro IPR001260