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mccc2

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Official Full Name
methylcrotonoyl-CoA carboxylase 2 (beta)
Background
This gene encodes the small subunit of3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, anautosomal recessive disorder of leucine catabolism.
Synonyms
MCCC2; methylcrotonoyl-CoA carboxylase 2 (beta); methylcrotonoyl Coenzyme A carboxylase 2 (beta); methylcrotonoyl-CoA carboxylase beta chain, mitochondrial; MCCB; biotin carboxylase; MCCase subunit beta; 3-methylcrotonyl-CoA carboxylase 2; methylcrotonoyl; methylcrotonoyl-Coenzyme A carboxylase 2 (beta); 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit; non-biotin containing subunit of 3-methylcrotonyl-CoA carboxylase; zgc:85685; si:dkey-57m14.1