MCCC1 is the large subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Defects in MCCC1 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 1 (MCC1 deficiency). MCC1 deficiency is an autosomal recessive disorder of leucine catabolism.
ab163430 on a 12.5% SDS-PAGE stained with Coomassie Blue.
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