Reaction: CPO transforms COPRO3 to PPGEN9
- in pathway: Heme biosynthesis
O2-dependent coproporpyrinogen oxidase (CPO) catalyzes the conversion of coproporphyrinogen III (COPRO3) to protoporphyrinogen IX (PPGEN9). The localization of the human enzyme to the mitochondrial intermembrane space is inferred from studies of the homologous rat enzyme (Elder and Evans 1978). The human enzyme functions as a homodimer (Lee et al. 2005). Enzyme deficiency is associated with hereditary coproporphyria in vivo.
Reaction - small molecule participants:
H2O2 [mitochondrial intermembrane space]
CO2 [mitochondrial intermembrane space]
PPGEN9 [mitochondrial intermembrane space]
O2 [mitochondrial intermembrane space]
COPRO3 [mitochondrial intermembrane space]
Reactome.org reaction link: R-HSA-189421
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Reaction input - small molecules:
dioxygen
coproporphyrinogen III
Reaction output - small molecules:
hydrogen peroxide
carbon dioxide
protoporphyrinogen
Reactome.org link: R-HSA-189421