ACACA

Acetyl-CoA carboxylase 1;ACC1;Acetyl-Coenzyme A carboxylase alpha;ACC-alpha;

ACACA ;
ACAC ;
ACC1 ;
ACCA ;
Acetyl-CoA carboxylase 1 ;
ACC-alpha ;

Acetyl-CoA carboxylase (ACC) is a complex multifunctional enzyme system. ACC is a biotin-containing enzyme which catalyzes the carboxylation of acetyl-CoA to malonyl-CoA , the rate-limiting step in fatty acid synthesis. There are two ACC forms , alpha and beta , encoded by two different genes. ACC-alpha is highly enriched in lipogenic tissues. The enzyme is under long term control at the transcriptional and translational levels and under short term regulation by the phosphorylation/dephosphorylation of targeted serine residues and by allosteric transformation by citrate or palmitoyl-CoA. Multiple alternatively spliced transcript variants divergent in the 5' sequence and encoding distinct isoforms have been found for this gene. [provided by RefSeq , Jul 2008] ,

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. ,

Cytoplasm , cytosol .

>>Fatty acid biosynthesis ;
>>Pyruvate metabolism ;
>>Propanoate metabolism ;
>>Metabolic pathways ;
>>Fatty acid metabolism ;
>>AMPK signaling pathway ;
>>Insulin signaling pathway ;
>>Glucagon signaling pathway ;
>>Alcoholic liver disease