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MGP000016

UniProt Annotations

Entry Information
Gene Nameacetyl-CoA carboxylase alpha
Protein EntryACACA_HUMAN
UniProt IDQ13085
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative promoter usage; Named isoforms=4; Name=1; IsoId=Q13085-1; Sequence=Displayed; Name=2; Synonyms=E5A; IsoId=Q13085-2; Sequence=VSP_026099; Name=3; Synonyms=E5B; IsoId=Q13085-3; Sequence=VSP_026098; Name=4; IsoId=Q13085-4; Sequence=VSP_026100;
Catalytic ActivityATP + acetyl-CoA + HCO(3)(-) = ADP + phosphate + malonyl-CoA. {ECO:0000269|PubMed:20952656}.
Catalytic ActivityATP + biotin-[carboxyl-carrier-protein] + CO(2) = ADP + phosphate + carboxy-biotin-[carboxyl-carrier- protein]. {ECO:0000269|PubMed:20952656}.
CofactorName=biotin; Xref=ChEBI:CHEBI:57586; Note=Biotin.;
CofactorName=Mn(2+); Xref=ChEBI:CHEBI:29035; Note=Binds 2 manganese ions per subunit.;
DiseaseAcetyl-CoA carboxylase 1 deficiency (ACACAD) [MIM:613933]: An inborn error of de novo fatty acid synthesis associated with severe brain damage, persistent myopathy and poor growth. {ECO:0000269|PubMed:6114432}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Enzyme RegulationBy phosphorylation (By similarity). Activity is increased by oligomerization. Citrate and MID1IP1 promote oligomerization. {ECO:0000250, ECO:0000269|PubMed:20952656}.
FunctionCatalyzes the rate-limiting reaction in the biogenesis of long-chain fatty acids. Carries out three functions: biotin carboxyl carrier protein, biotin carboxylase and carboxyltransferase. {ECO:0000269|PubMed:20952656}.
InteractionO60218: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;
PathwayLipid metabolism; malonyl-CoA biosynthesis; malonyl-CoA from acetyl-CoA: step 1/1.
PtmPhosphorylation on Ser-1263 is required for interaction with BRCA1. {ECO:0000269|PubMed:16698035, ECO:0000269|PubMed:17081983, ECO:0000269|PubMed:18220336, ECO:0000269|PubMed:18669648, ECO:0000269|PubMed:18691976, ECO:0000269|PubMed:19369195, ECO:0000269|PubMed:19690332, ECO:0000269|PubMed:20068231, ECO:0000269|PubMed:21406692, ECO:0000269|Ref.7}.
Sequence CautionSequence=AAP94120.1; Type=Erroneous initiation; Evidence={ECO:0000305};
SimilarityContains 1 ATP-grasp domain. {ECO:0000255|PROSITE- ProRule:PRU00409}.
SimilarityContains 1 biotin carboxylation domain. {ECO:0000305}.
SimilarityContains 1 biotinyl-binding domain. {ECO:0000255|PROSITE-ProRule:PRU01066, ECO:0000305}.
SimilarityContains 1 carboxyltransferase domain. {ECO:0000305}.
Subcellular LocationCytoplasm.
SubunitMonomer, 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. {ECO:0000269|PubMed:12360400, ECO:0000269|PubMed:16326698, ECO:0000269|PubMed:20952656}.
Tissue SpecificityExpressed in brain, placental, skeletal muscle, renal, pancreatic and adipose tissues; expressed at low level in pulmonary tissue; not detected in the liver.
Web ResourceName=Wikipedia; Note=Acetyl-CoA carboxylase entry; URL="http://en.wikipedia.org/wiki/Acetyl-CoA_carboxylase";
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