Catalytic activity:ATP + acetyl-CoA + HCO (3) (-) = ADP + phosphate + malonyl-CoA. ,Catalytic activity:ATP + biotin-carboxyl-carrier protein + CO (2) = ADP + phosphate + carboxybiotin-carboxyl-carrier protein. ,cofactor:Binds 2 manganese ions per subunit. ,cofactor:Biotin. ,Disease:Defects in ACACA are a cause of ACACA deficiency [MIM:200350]; also called ACAC or ACC deficiency. ACACA deficiency is an inborn error of de novo fatty acid synthesis. The disorder is associated with severe brain damage , persistent myopathy and poor growth. ,enzyme regulation:By phosphorylation. ,Function:Catalyzes the rate-limiting reaction in the biogenesis of long-chain fatty acids. Carries out three functions: biotin carboxyl carrier protein , biotin carboxylase and carboxyltransferase. ,online information:Acetyl-CoA carboxylase entry ,pathway:Lipid metabolism; malonyl-CoA biosynthesis; malonyl-CoA from acetyl-CoA: step 1/1. ,PTM:Phosphorylation on Ser-1263 is required for interaction with BRCA1. ,similarity:Contains 1 ATP-grasp domain. ,similarity:Contains 1 biotin carboxylation domain. ,similarity:Contains 1 biotinyl-binding domain. ,similarity:Contains 1 carboxyltransferase domain. ,subunit:Interacts in its inactive phosphorylated form with the BRCT domains of BRCA1 which prevents ACACA dephosphorylation and inhibits lipid synthesis. ,tissue specificity:Expressed in brain , placental , skeletal muscle , renal , pancreatic and adipose tissues; expressed at low level in pulmonary tissue; not detected in the liver. ,
展开内容