3-methylcrotonyl-CoA carboxylase-beta (3-methylcrotonyl-CoA:carbon dioxide ligase beta subunit, 3-Methylcrotonyl-CoA carboxylase 2)

Function

• 3-methylcrotonyl-CoA carboxylase beta subunit
• leucine catabolism

Compartment

mitochondrial matrix

Pathology

• defects in MCCC2 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 2

More general terms

• protein subunit
• mitochondrial protein

Component of

• 3-methylcrotonyl-CoA carboxylase (MCCase, 3-methylcrotonyl-CoA:carbon dioxide ligase)

Database

• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:6408
• OMIM: https://mirror.omim.org/entry/210210
• UniProt: http://www.uniprot.org/uniprot/Q9HCC0.html