Methylcrotonyl CoA carboxylase (MCC) (3-Methylcrotonyl CoA carboxylase methylcrotonoyl-CoA carboxylase) is a biotin-requiring enzyme located in the mitochondria. MCC uses bicarbonate as a carboxyl group source to catalyze the carboxylation of a carbon adjacent to a carbonyl group performing the 4th step in processing leucine an essential amino acid.
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