The protein encoded by this gene is the sixth enzyme of the heme biosynthetic pathway. The encoded enzyme is soluble and found in the intermembrane space of mitochondria. This enzyme catalyzes the stepwise oxidative decarboxylation of coproporphyrinogen III to protoporphyrinogen IX, a precursor of heme. Defects in this gene are a cause of hereditary coproporphyria (HCP).
Categories
Primary Antibodies
Clonality
polyclonal
Description
CPOX(Coproporphyrinogen-III oxidase, mitochondrial) is also named as CPO and CPX. Human CPO is a 76 kDa protein that is active as a homodimer in the mitochondrial intermembrane pace(PMID:16159891).Mutations in human CPO gene predict the clinical outcome of the disease, with either hepatic hereditary coproporphyria or hematological manifestations of erythropoietic harderoporphyria.CPOX has a transit peptide of 110 aa and the chain contains 344 aa.