Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

Official Name

acetyl-CoA carboxylase

Name from literature

acetyl CoA carboxylase

Pathway from literature

the de novo synthesis of lipids and cholesterol

Pathway from KEGG

Biosynthesis of Secondary Metabolites; Tetracycline biosynthesis; map00253

Cellular Processes; Endocrine System; Insulin signaling pathway; map04910

Carbohydrate Metabolism; Pyruvate metabolism; map00620

Carbohydrate Metabolism; Propanoate metabolism; map00640

Lipid Metabolism; Fatty acid biosynthesis; map00061


Human (9606)

Genome localization

17q21[31 ], 12q24.11[32 ],


A biotinyl-protein. Also catalyses transcarboxylation; the plant enzyme also carboxylates propanonyl-CoA and butanoyl-CoA.

Rate-limiting Description

"At high (supraphysiological) concentrations of GLA, the specific downregulation of FAS gene expression leads to accumulation of the substrate for FAS, malonyl-CoA, that, as a result of FAS blockade, continue to be generated by the rate-limiting enzyme of the fatty acid biosynthetic pathway acetyl-CoA carboxilase, which is not inhibited in the absence of FAS-catalyzed long chain endogenous fatty acids." (15607568)

Regulatory Information

Upstream transcription factor


Regulatory type


transcriptional factor;REST(5978)

"This sequence census method was then used to map in vivo binding of the neuron-restrictive silencer factor (NRSF; also known as REST, for repressor element-1 silencing transcription factor) to 1946 locations in the human genome." (17540862)


"Transcription of ACC-alpha from at least three promoters and the potential to generate ACC-alpha isozymes with differential susceptibilities to phosphorylation indicate that the regulation of fatty acid synthesis in human tissues is likely to be complex" (14643797)


"the whole BRCA1 protein interacts with ACCA when phosphorylated on Ser1263." (16698035)


"elevation of AMPK via phosphorylation is not sufficient to maintain elevated ACCbeta Ser(221) phosphorylation during exercise" (12413941)







Gene ontology

Gene ontology

GO:0030145 (F) manganese ion binding [Q13085, O00763 ];
GO:0016020 (C) membrane [O00763 ];
GO:0012505 (C) endomembrane system [O00763 ];
GO:0009374 (F) biotin binding [Q13085, O00763 ];
GO:0004075 (F) biotin carboxylase activity [Q13085, O00763 ];
GO:0003989 (F) acetyl-CoA carboxylase activity [Q13085, O00763 ];
GO:0005737 (C) cytoplasm [Q13085 ];
GO:0005829 (C) cytosol [O00763 ];
GO:0006633 (P) fatty acid biosynthetic process [Q13085, O00763 ];
GO:0005794 (C) Golgi apparatus [O00763 ];
GO:0005524 (F) ATP binding [Q13085, O00763 ];
GO:0046320 (P) regulation of fatty acid oxidation [O00763 ];
GO:0005515 (F) protein binding [Q13085 ];

Tissue expression

Tissue From HPRD

Thymus [01938 ];
Salivary gland [01938 ];
Testis [01938 ];
Skeletal muscle [01938, 07044 ];
Liver [01938, 07044 ];
Muscle [01938 ];
Brain [01938 ];
Prostate [01938 ];
Adipose tissue [01938 ];
Kidney [01938 ];
Adrenal gland [01938 ];
Mammary gland [01938 ];
Lung [01938 ];
Heart [01938, 07044 ];
Pancreas [01938 ];
Cerebellum [01938 ];
Placenta [01938 ];

Tissue specificity

Predominantly expressed in the heart, skeletal muscles and liver [O00763 ];

Expressed in brain, placental, skeletal muscle, renal, pancreatic and adipose tissues;
expressed at low level in pulmonary tissue;
not detected in the liver [Q13085 ];

Subcellular localization


membrane [O00763 ];

cytoplasm [Q13085 ];

Disease relevance


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 [Q13085 ];



O00763; Q13085

Entrez Gene

31; 32


01938; 07044

  Copyright 2009, Center for Bioinformatics 
  Last Modified: 2009-03-24  
  Design by Zhao Min