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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).
仅用于科研。不用于诊断过程。未经明确授权不得转售。
蛋白别名: coprogen oxidase; coproporphyrinogen-III oxidase, mitochondrial; coproporphyrinogenase; COX; Oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
基因别名: CPO; CPOX; CPX; HCP
UniProt ID: (Human) P36551
Entrez Gene ID: (Human) 1371