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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 results in a reduced production of heme in animals. The medical condition associated with this enzyme defect is called hereditary coproporphyria. CPOX, the sixth enzyme of the haem biosynthetic pathway, converts coproporphyrinogen III to protoporphyrinogen IX through two sequential steps of oxidative decarboxylation. The activity of the CPOX enzyme, located in the mitochondrial membrane, is measured in lymphocytes.

AttributesValues
rdf:type
rdfs:label
  • Coproporphyrinogen-Oxidase (de)
  • Coproporphyrinogen III oxidase (en)
  • Coproporfirinogeno ossidasi (it)
  • コプロポルフィリノーゲンオキシダーゼ (ja)
rdfs:comment
  • 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 results in a reduced production of heme in animals. The medical condition associated with this enzyme defect is called hereditary coproporphyria. CPOX, the sixth enzyme of the haem biosynthetic pathway, converts coproporphyrinogen III to protoporphyrinogen IX through two sequential steps of oxidative decarboxylation. The activity of the CPOX enzyme, located in the mitochondrial membrane, is measured in lymphocytes. (en)
  • Coproporphyrinogen-Oxidase (CPOX) ist das Enzym, das die Oxidation von zu Protoporphyrinogen IX katalysiert, eine Teilreaktion bei der Biosynthese der Porphyrine, die in allen Lebewesen stattfindet. In Eukaryoten wird vorher das Substrat aus dem Zytosol in den Raum zwischen der inneren und äußeren Membran der Mitochondrien transportiert, der beteiligte Transporter ist noch unbekannt. Beim Menschen können Mutationen am CPOX-Gen zu CPOX-Mangel und damit zu hereditärer Koproporphyrie führen. (de)
  • La coproporfirinogeno ossidasi è un enzima appartenente alla classe delle ossidoreduttasi, che catalizza la seguente reazione: copro III + O2 + 2 H+ ⇄ protoporfirinogeno-IX + 2 CO2 + 2 H2O (it)
  • コプロポルフィリノーゲンオキシダーゼ（coproporphyrinogen oxidase）は、ヒトのCPOX遺伝子にコードされている酵素である。この酵素はポルフィリン代謝の6番目の反応に関与し、コプロポルフィリノーゲンIIIをプロトポルフィリノーゲンIXに変換する。 * コプロポルフィリノーゲンIII * プロトポルフィリノーゲンIX (ja)
name
  • Coprogen_oxidas (en)
foaf:depiction
  • http://commons.wikimedia.org/wiki/Special:FilePath/PDB_1vju_EBI.jpg
  • http://commons.wikimedia.org/wiki/Special:FilePath/Heme_synthesis.png
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PROSITE
  • PDOC00783 (en)
caption
  • coproporphyrinogen iii oxidase from leishmania major (en)
InterPro
  • IPR001260 (en)
Pfam
  • PF01218 (en)
symbol
  • Coprogen_oxidas (en)
has abstract
  • 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 results in a reduced production of heme in animals. The medical condition associated with this enzyme defect is called hereditary coproporphyria. CPOX, the sixth enzyme of the haem biosynthetic pathway, converts coproporphyrinogen III to protoporphyrinogen IX through two sequential steps of oxidative decarboxylation. The activity of the CPOX enzyme, located in the mitochondrial membrane, is measured in lymphocytes. (en)
  • Coproporphyrinogen-Oxidase (CPOX) ist das Enzym, das die Oxidation von zu Protoporphyrinogen IX katalysiert, eine Teilreaktion bei der Biosynthese der Porphyrine, die in allen Lebewesen stattfindet. In Eukaryoten wird vorher das Substrat aus dem Zytosol in den Raum zwischen der inneren und äußeren Membran der Mitochondrien transportiert, der beteiligte Transporter ist noch unbekannt. Beim Menschen können Mutationen am CPOX-Gen zu CPOX-Mangel und damit zu hereditärer Koproporphyrie führen. (de)
  • La coproporfirinogeno ossidasi è un enzima appartenente alla classe delle ossidoreduttasi, che catalizza la seguente reazione: copro III + O2 + 2 H+ ⇄ protoporfirinogeno-IX + 2 CO2 + 2 H2O (it)
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