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MMUT function in mitochondria. Propionate formed from the catabolism of branched amino acids and odd-chain fatty acids is converted to D-methylmalonyl-CoA. Methylmanolyl-CoA epimerase isomerizes D-methylmalonyl-CoA to the L-form. L-methylmalonyl-CoA is the substrate for MMUT, converting it to succinyl-CoA, which enters the TCA cycle. Abbreviations: MMA—methylmalonic acid, TCA cycle—the tricarboxylic acid cycle.
