Aliases

• 3-methylcrotonyl-CoA carboxylase 2
• 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit
• 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta
• EC 6.4.1.4
• MCCB
• MCCC2
• MCCase subunit beta
• biotin carboxylase
• methylcrotonoyl-CoA carboxylase 2 (beta)
• methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
• methylcrotonoyl-Coenzyme A carboxylase 2 (beta)
• non-biotin containing subunit of 3-methylcrotonyl-CoA carboxylase

Description

From NCBI Gene: This gene encodes the small 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. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, May 2018]

Attributes

QA State

Under Review

Type

Gene

HGNC Name

MCCC2