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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)