MCCC2
MCCC2 (methylcrotonyl-CoA carboxylase subunit 2, also known as MCCB or MCCCβ) is the carboxyltransferase subunit of 3-methylcrotonyl-CoA carboxylase. It supports a critical step in the mitochondrial catabolism of leucine and isovaleric acid. Browse Assay Genie's current range of MCCC2 research reagents below, or read on for the biology behind the target.
MCCC2 is the carboxyltransferase subunit of 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyses the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA. This reaction is a critical step in the catabolism of leucine and isovaleric acid. The enzyme functions as a heterodimer, and the MCCC2 gene encodes its small subunit. The protein is located in the mitochondrial matrix and is classified under EC 6.4.1.4.
The MCCC2 protein, also called methylcrotonoyl-CoA carboxylase beta chain, belongs to the AccD/PCCB family. HGNC places the gene in the group of methylcrotonyl-CoA carboxylase subunits. Alternative splicing of the MCCC2 gene results in multiple transcript variants that encode distinct isoforms. Orthologues are annotated in both mouse and rat, where the gene is named Mccc2.
Earlier names for the gene include methylcrotonoyl-Coenzyme A carboxylase 2 (beta), and the symbol MCCB is still used as an alias. Mutations in MCCC2 are associated with 3-methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. This link makes the gene a useful reference point for studies of branched-chain amino acid breakdown and inherited metabolic disease.
MCCC2 contributes to a critical step in leucine and isovaleric acid catabolism, making it relevant to studies of branched-chain amino acid metabolism.
Mutations in MCCC2 are associated with 3-methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism.
As the carboxyltransferase subunit of a heterodimeric mitochondrial matrix enzyme, MCCC2 is relevant to work on biotin-dependent carboxylase function.
Alternative splicing yields multiple transcript variants encoding distinct isoforms, which supports investigation of how MCCC2 expression is regulated.
MCCC2 is the carboxyltransferase subunit of 3-methylcrotonyl-CoA carboxylase. The enzyme converts 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step in leucine and isovaleric acid catabolism. The enzyme works as a heterodimer in the mitochondrial matrix, and MCCC2 encodes its small subunit.
MCCC2 is also written MCCB or MCCCβ, and its UniProt name is methylcrotonoyl-CoA carboxylase beta chain, mitochondrial. Older names include methylcrotonoyl-Coenzyme A carboxylase 2 (beta). Alternative splicing produces multiple transcript variants that encode distinct isoforms.
Mutations in MCCC2 are associated with 3-methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. Mouse and rat both carry an orthologous gene, Mccc2. Check the datasheet of any product for its validated reactivity.
Other carboxylase subunits and leucine catabolism enzymes studied alongside MCCC2.
Gene and protein annotations from HGNC, NCBI Gene and UniProt Q9HCC0; product specifications from Assay Genie datasheets.