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Methylcrotonyl-CoA carboxylase
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 fourth step in processing leucine, an essential amino acid.〔Bruice, Paula Y. Organic Chemistry. New Jersey: Prentice Hall, 2001. 1010-1011.〕
== Function ==
During branched-chain amino acid degradation, MCC performs a single step in the breakdown of leucine to eventually yield acetyl CoA and acetoacetate.〔Berg, Jeremy M., John L. Tymoczko, Lubert Stryer. Biochemistry. New York: Freeman, 2002. 652-653.〕 MCC catalyzes the carboxylation of 3-methylcrotonyl CoA to 3-methylglutaconyl CoA. 3-Methylglutaconyl CoA is then hydrated to produce 3-hydroxy-3-methylglutaryl CoA. 3-Hydroxy-3-methylglutaryl CoA is cleaved into two molecules, acetoacetate and acetyl CoA.
Point mutations and deletion events in the genes coding for MCC can lead to MCC deficiency, an inborn error of metabolism which usually presents with vomiting, metabolic acidosis, very low plasma glucose concentration, and very low levels of carnitine in plasma.〔Stipanuk, Martha H. Biochemical and Physiological Aspects of Human Nutrition. New York: Saunders, 2000. 535-536.〕
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