3-methylcrotonyl-CoA carboxylase-alpha (3-methylcrotonyl-CoA:carbon dioxide ligase alpha subunit, 3-methylcrotonyl-CoA carboxylase 1, MCCase subunit alpha, 3- methylcrotonyl-CoA carboxylase biotin-containing subunit, MCCC1, MCCA)

Function

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

Cofactor: biotin

Structure

• contains 1 ATP-grasp domain
• contains 1 biotin carboxylation domain
• contains 1 biotinyl-binding domain

Compartment

mitochondrial matrix

Pathology

• defects in MCCC1 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 1

More general terms

• protein subunit
• mitochondrial protein

Component of

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

Database

• OMIM: https://mirror.omim.org/entry/210200
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:5692
• UniProt: http://www.uniprot.org/uniprot/Q96RQ3.html