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In mitochondria, the oxidized or Co(III) form of vitamin B12, namely, 5′-deoxyadenosylcobalamin, is required for the mitochondrial enzyme methylmalonyl-CoA mutase to convert methylmalonyl-CoA to succinyl-CoA, which is important in hemoglobin synthesis, the oxidation of odd-chain fatty acids, and the catabolism of ketogenic amino acids [6, 7]
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