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3-methylcrotonyl-CoA carboxylase (MCCase, 3-methylcrotonyl-CoA:carbon dioxide ligase)
Function:
ATP + 3-methylcrotonyl-CoA + HCO3-
ADP + phosphate + 3-methylglutaconyl-CoA
Structure:
- probably a complex of 6 biotin-containing alpha subunits & 6 beta subunits
Laboratory:
- 3-methylcrotonyl-CoA carboxylase in fibroblasts
General
mitochondrial protein
multisubunit protein
Properties
COMPARTMENT: mitochondrial matrix
SUBUNITS: 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) (6)
MOTIF: Biotin carboxylation {48-494}
MOTIF: ATP-binding site
NAME: ATP-binding site
SITE: 163-163
ATP-grasp domain
SITE: 167-364
ATP-binding site
NAME: ATP-binding site
SITE: 247-247
ATP-binding site
NAME: ATP-binding site
SITE: 282-282
arginine residue {R339}
binding site
SITE: 629-714
FOR-BINDING-OF: biotin
MOTIF: binding site
SITE: 681-681
FOR-BINDING-OF: biotin
3-methylcrotonyl-CoA carboxylase-beta (6)
MOTIF: binding site
SITE: 343-372
FOR-BINDING-OF: acyl Coenzyme A
Database Correlations
ENZYME 6.4.1.4
References
UniProt :accession Q9HCC0
Components
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)
3-methylcrotonyl-CoA carboxylase-beta (3-methylcrotonyl-CoA:carbon dioxide ligase beta subunit, 3-Methylcrotonyl-CoA carboxylase 2)