ID:ACACA_HUMAN DESCRIPTION: RecName: Full=Acetyl-CoA carboxylase 1; Short=ACC1; EC=6.4.1.2; AltName: Full=ACC-alpha; Includes: RecName: Full=Biotin carboxylase; EC=6.3.4.14; 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. CATALYTIC ACTIVITY: ATP + acetyl-CoA + HCO(3)(-) = ADP + phosphate + malonyl-CoA. CATALYTIC ACTIVITY: ATP + biotin-[carboxyl-carrier-protein] + CO(2) = ADP + phosphate + carboxy-biotin-[carboxyl-carrier- protein]. COFACTOR: Biotin. COFACTOR: Binds 2 manganese ions per subunit. ENZYME REGULATION: By phosphorylation (By similarity). Activity is increased by oligomerization. Citrate and MID1IP1 promote oligomerization. PATHWAY: Lipid metabolism; malonyl-CoA biosynthesis; malonyl-CoA from acetyl-CoA: step 1/1. SUBUNIT: Monomer, homodimer, and homotetramer. Can form filamentous polymers. Interacts in its inactive phosphorylated form with the BRCT domains of BRCA1 which prevents ACACA dephosphorylation and inhibits lipid synthesis. Interacts with MID1IP1; interaction with MID1IP1 promotes oligomerization and increases its activity. INTERACTION: O60218:AKR1B10; NbExp=4; IntAct=EBI-717681, EBI-1572139; P38398:BRCA1; NbExp=2; IntAct=EBI-717681, EBI-349905; Q96EB6:SIRT1; NbExp=3; IntAct=EBI-717681, EBI-1802965; SUBCELLULAR LOCATION: Cytoplasm. 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. PTM: Phosphorylation on Ser-1263 is required for interaction with BRCA1. DISEASE: Defects in ACACA are a cause of acetyl-CoA carboxylase 1 deficiency (ACACAD) [MIM:613933]; also known as ACAC deficiency or ACC deficiency. An inborn error of de novo fatty acid synthesis associated with severe brain damage, persistent myopathy and poor growth. SIMILARITY: Contains 1 ATP-grasp domain. SIMILARITY: Contains 1 biotin carboxylation domain. SIMILARITY: Contains 1 biotinyl-binding domain. SIMILARITY: Contains 1 carboxyltransferase domain. SEQUENCE CAUTION: Sequence=AAP94120.1; Type=Erroneous initiation; WEB RESOURCE: Name=Wikipedia; Note=Acetyl-CoA carboxylase entry; URL="http://en.wikipedia.org/wiki/Acetyl-CoA_carboxylase";
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q13085
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0001894 tissue homeostasis GO:0006084 acetyl-CoA metabolic process GO:0006629 lipid metabolic process GO:0006631 fatty acid metabolic process GO:0006633 fatty acid biosynthetic process GO:0006853 carnitine shuttle GO:0008152 metabolic process GO:0019538 protein metabolic process GO:0031325 positive regulation of cellular metabolic process GO:0045540 regulation of cholesterol biosynthetic process GO:0046949 fatty-acyl-CoA biosynthetic process GO:0051289 protein homotetramerization GO:0055088 lipid homeostasis GO:0071380 cellular response to prostaglandin E stimulus GO:2001295 malonyl-CoA biosynthetic process
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
