Coproporphyrinogen Oxidase

From Anvita Health Wiki

Contents 1 Function 2 Structure 3 Compartment 4 Pathology 5 More General Terms 6 Additional Terms 7 Internet Database 8 References

Function

• key enzyme in heme biosynthesis
• catalyzes oxidative decarboxylation of propionic acid side chains of rings A & B of coproporphyrinogen III
• porphyrin metabolism; protoporphyrin-IX biosynthesis
• protoporphyrinogen-IX from coproporphyrinogen-III (O2 route): step 1/1
  

• Coproporphyrinogen-III + O2 + 2 H+ = protoporphyrinogen-IX + 2 CO2 + 2 H2O

Structure

• homodimer
• belongs to the aerobic coproporphyrinogen-III oxidase family

Compartment

• mitochondria intermembrane space

Pathology

• defects in CPOX are the cause of hereditary coproporphyria

More General Terms

• oxidoreductase
• membrane protein
• mitochondrial protein

Additional Terms

• heme synthesis

Internet Database

Entrez gene: 1371
Kegg: [1]
OMIM: 121300
OMIM: 612732
Prosite: [2]
UniProt: [3]

References

1. UniProt [4]
2. GeneReviews [5]

Coproporphyrinogen-III oxidase, mitochondrial; COX; Coprogen oxidase; Coproporphyrinogenase (CPOX, CPO, CPX)