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Coproporphyrinogen III oxidase information


CPOX
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesCPOX, CPO, CPX, HCP, coproporphyrinogen oxidase, COX, HARPO
External IDsOMIM: 612732; MGI: 104841; HomoloGene: 76; GeneCards: CPOX; OMA:CPOX - orthologs
EC number1.3.3.3
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_000097

NM_007757

RefSeq (protein)

NP_000088

NP_031783

Location (UCSC)n/aChr 16: 58.49 – 58.54 Mb
PubMed search[2][3]
Wikidata
View/Edit HumanView/Edit Mouse
Coprogen oxidase
coproporphyrinogen iii oxidase from leishmania major
Identifiers
SymbolCoprogen oxidase
PfamPF01218
InterProIPR001260
PROSITEPDOC00783
Available protein structures:
Pfam  structures / ECOD  
PDBRCSB PDB; PDBe; PDBj
PDBsumstructure summary

Coproporphyrinogen-III oxidase, mitochondrial (abbreviated as CPOX) is an enzyme that in humans is encoded by the CPOX gene.[4][5][6] 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.[7][8]

CPOX, the sixth enzyme of the haem biosynthetic pathway, converts coproporphyrinogen III to protoporphyrinogen IX through two sequential steps of oxidative decarboxylation.[9] The activity of the CPOX enzyme, located in the mitochondrial membrane, is measured in lymphocytes.[10]

  1. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000022742 – Ensembl, May 2017
  2. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  3. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ Lamoril J, Martasek P, Deybach JC, Da Silva V, Grandchamp B, Nordmann Y (February 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.
  5. ^ Kohno H, Furukawa T, Yoshinaga T, Tokunaga R, Taketani S (October 1993). "Coproporphyrinogen oxidase. Purification, molecular cloning, and induction of mRNA during erythroid differentiation". The Journal of Biological Chemistry. 268 (28): 21359–63. doi:10.1016/S0021-9258(19)36931-5. PMID 8407975.
  6. ^ "Entrez Gene: CPOX coproporphyrinogen oxidase".
  7. ^ "Hereditary coproporphyria". Genetic and Rare Diseases Information Center. National Institutes of Health. Archived from the original on 7 August 2012. Retrieved 8 August 2011.
  8. ^ "CPOX". Genetics Home Reference. Retrieved 8 August 2011.
  9. ^ Sano S, Granick S (April 1961). "Mitochondrial coproporphyrinogen oxidase and protoporphyrin formation". The Journal of Biological Chemistry. 236 (4): 1173–80. doi:10.1016/S0021-9258(18)64262-0. PMID 13746277.
  10. ^ Guo R, Lim CK, Peters TJ (October 1988). "Accurate and specific HPLC assay of coproporphyrinogen III oxidase activity in human peripheral leucocytes". Clinica Chimica Acta; International Journal of Clinical Chemistry. 177 (3): 245–52. doi:10.1016/0009-8981(88)90069-1. PMID 3233772.

and 18 Related for: Coproporphyrinogen III oxidase information

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Coproporphyrinogen III oxidase

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Coproporphyrinogen-III oxidase, mitochondrial (abbreviated as CPOX) is an enzyme that in humans is encoded by the CPOX gene. A genetic defect in the enzyme...

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Coproporphyrinogen III

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carbon dioxide molecules. Coproporphyrinogen III is further used as a substrate for the enzyme coproporphyrinogen III oxidase which oxidizes and further...

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Coproporphyrinogens

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uroporphyrinogen III by the enzyme uroporphyrinogen III decarboxylase, and it is converted into protoporphyrinogen IX by coproporphyrinogen III oxidase. Coproporphyrinogens...

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Porphyria

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Hereditary coproporphyria, which is characterized by a deficiency in coproporphyrinogen oxidase, coded for by the CPOX gene, may also present with both acute...

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Coproporphyrinogen dehydrogenase

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include oxygen-independent coproporphyrinogen-III oxidase, HemF, HemN, radical SAM enzyme, and coproporphyrinogen III oxidase. This enzyme participates...

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Heme

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G, Biolcati G (February 2009). "Four novel mutations of the coproporphyrinogen III oxidase gene". Cellular and Molecular Biology. 55 (1): 8–15. PMID 19267996...

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Porphyrin

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which is hydrolysed to form the circular tetrapyrrole uroporphyrinogen III. This molecule undergoes a number of further modifications. Intermediates...

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Radical SAM enzymes

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or anaerobic coproporphyrinogen III oxidase is a radical SAM enzyme that catalyzes the oxidative decarboxylation of coproporphyrinogen III to protoporhyrinogen...

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Metalysinibacillus

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arginine-binding extracellular protein ArtP precursor, oxygen-independent coproporphyrinogen III oxidase, putative hydrolase MhqD, helix-turn-helix transcriptional regulator...

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CPOX

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CPOX may refer to: Coproporphyrinogen III oxidase Catalytic partial oxidation This disambiguation page lists articles associated with the title CPOX. If...

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Methyltransferase

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Class C methylase has homologous sequence with the RS enzyme, coproporphyrinogen III oxidase (HemN), which also catalyzes the methylation of sp2-hybridized...

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Hereditary coproporphyria

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hepatic porphyria. HCP is caused by a deficiency of the enzyme coproporphyrinogen oxidase, coded for by the CPOX gene, and is inherited in an autosomal...

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Protoporphyrinogen oxidase

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anemia) CPOX: coproporphyrinogen oxidase FECH: ferrochelatase (protoporphyria) HMBS: hydroxymethylbilane synthase PPOX: protoporphyrinogen oxidase UROD: uroporphyrinogen...

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Protoporphyrinogen IX

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compound is synthesized in most organisms from coproporphyrinogen III by the enzyme coproporphyrinogen oxidase: The process entails conversion of two of four...

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Porphyrinogen

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in the methylene bridges). Uroporphyrinogen III, precursor to coproporphyrinogen III. coproporphyrinogen III, precursor to protoporphyrinogen IX. Protoporphyrinogen...

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Harderoporphyria

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other porphyria. Enzyme tests show markedly reduced activity of coproporphyrinogen oxidase, compared to both unaffected individuals and those affected with...

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Leghemoglobin

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O, Sandal L, Sandal NN, Marcker KA (October 1993). "A soybean coproporphyrinogen oxidase gene is highly expressed in root nodules". Plant Molecular Biology...

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Index of molecular biology articles

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consensus sequence - conservative substitution - conserved - contig - coproporphyrinogen dehydrogenase - cortisone alpha-reductase - cosmid - costunolide synthase...

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