CPO transforms COPRO3 to PPGEN9

Stable Identifier
R-HSA-189421
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
Conversion of coproporphyrinogen III to protoporphyrinogen IX
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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.

Literature References
PubMed ID Title Journal Year
16258391 Kinetic evaluation of human cloned coproporphyrinogen oxidase using a ring isomer of the natural substrate

Cooper, CL, Lash, TD, Jones, MA

Med Sci Monit 2005
666752 Evidence that the coproporphyrinogen oxidase activity of rat liver is situated in the intermembrane space of mitochondria

Elder, GH, Evans, JO

Biochem J 1978
16176984 Structural basis of hereditary coproporphyria

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

Proc Natl Acad Sci U S A 2005
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
coproporphyrinogen oxidase activity of 2xCPO [mitochondrial intermembrane space]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created