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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).
cac; clone 560; coprogen oxidase; Coproporphyrinogen III oxidase, mitochondrial precursor (Coproporphyrinogenase) (Coprogen oxidase) (COX); coproporphyrinogen oxidase; coproporphyrinogenase; coproporphyrinogen-III oxidase, mitochondrial; COX; CPO; Cpox; cpox {ECO:0000312; CPX; EMBL:AAI07665.1}; HCP; nct; oxygen-dependent coproporphyrinogen-III oxidase, mitochondrial
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100 µL
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