CPO transforms COPRO3 to PPGEN9

Stable Identifier
Reaction [transition]
Homo sapiens
coproporphyrinogen III + 2 H(+) + O2 => 2 CO2 + 2 H2O + protoporphyrinogen IX, Conversion of coproporphyrinogen III to protoporphyrinogen IX
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O2-dependent coproporpyrinogen oxidase (CPO) catalyzes the conversion of coproporphyrinogen III (COPRO3) to protoporphyrinogen IX (PPGEN9) (Martasek et al. 1994). 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.
Literature References
PubMed ID Title Journal Year
8159699 Molecular cloning, sequencing, and functional expression of a cDNA encoding human coproporphyrinogen oxidase

Grandchamp, B, Labbe, P, Dumas, JB, de Verneuil, H, Delfau-Larue, MH, Montagne, JJ, Martasek, P, Camadro, JM

Proc Natl Acad Sci U S A 1994
666752 Evidence that the coproporphyrinogen oxidase activity of rat liver is situated in the intermembrane space of mitochondria

Evans, JO, Elder, GH

Biochem J 1978
16258391 Kinetic evaluation of human cloned coproporphyrinogen oxidase using a ring isomer of the natural substrate

Cooper, CL, Jones, MA, Lash, TD

Med Sci Monit 2005
16176984 Structural basis of hereditary coproporphyria

Lee, DS, Martasek, P, Flachsova, E, Demeler, B, Bodnarova, M, Raman, CS

Proc Natl Acad Sci U S A 2005
Catalyst Activity

coproporphyrinogen oxidase activity of 2xCPO [mitochondrial intermembrane space]

Orthologous Events
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