Names & Taxonomy
- Uniprot ID:
- O00763
- Entry Name:
- ACACB_HUMAN
- Status:
- reviewed
- Protein Names:
- Acetyl-CoA carboxylase 2 (EC 6.4.1.2) (ACC-beta) [Includes: Biotin carboxylase (EC 6.3.4.14)]
- Gene Names:
- ACACB ACC2 ACCB
- Gene Names Primary:
- ACACB
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 2458
- Sequence:
- MVLLLCLSCLIFSCLTFSWLKIWGKMTDSKPITKSKSEANLIPSQEPFPASDNSGETPQRNGEGHTLPKTPSQAEPASHKGPKDAGRRRNSLPPSHQKPPRNPLSSSDAAPSPELQANGTGTQGLEATDTNGLSSSARPQGQQAGSPSKEDKKQANIKRQLMTNFILGSFDDYSSDEDSVAGSSRESTRKGSRASLGALSLEAYLTTGEAETRVPTMRPSMSGLHLVKRGREHKKLDLHRDFTVASPAEFVTRFGGDRVIEKVLIANNGIAAVKCMRSIRRWAYEMFRNERAIRFVVMVTPEDLKANAEYIKMADHYVPVPGGPNNNNYANVELIVDIAKRIPVQAVWAGWGHASENPKLPELLCKNGVAFLGPPSEAMWALGDKIASTVVAQTLQVPTLPWSGSGLTVEWTEDDLQQGKRISVPEDVYDKGCVKDVDEGLEAAERIGFPLMIKASEGGGGKGIRKAESAEDFPILFRQVQSEIPGSPIFLMKLAQHARHLEVQILADQYGNAVSLFGRDCSIQRRHQKIVEEAPATIAPLAIFEFMEQCAIRLAKTVGYVSAGTVEYLYSQDGSFHFLELNPRLQVEHPCTEMIADVNLPAAQLQIAMGVPLHRLKDIRLLYGESPWGVTPISFETPSNPPLARGHVIAARITSENPDEGFKPSSGTVQELNFRSSKNVWGYFSVAATGGLHEFADSQFGHCFSWGENREEAISNMVVALKELSIRGDFRTTVEYLINLLETESFQNNDIDTGWLDYLIAEKVQAEKPDIMLGVVCGALNVADAMFRTCMTDFLHSLERGQVLPADSLLNLVDVELIYGGVKYILKVARQSLTMFVLIMNGCHIEIDAHRLNDGGLLLSYNGNSYTTYMKEEVDSYRITIGNKTCVFEKENDPTVLRSPSAGKLTQYTVEDGGHVEAGSSYAEMEVMKMIMTLNVQERGRVKYIKRPGAVLEAGCVVARLELDDPSKVHPAEPFTGELPAQQTLPILGEKLHQVFHSVLENLTNVMSGFCLPEPVFSIKLKEWVQKLMMTLRHPSLPLLELQEIMTSVAGRIPAPVEKSVRRVMAQYASNITSVLCQFPSQQIATILDCHAATLQRKADREVFFINTQSIVQLVQRYRSGIRGYMKTVVLDLLRRYLRVEHHFQQAHYDKCVINLREQFKPDMSQVLDCIFSHAQVAKKNQLVIMLIDELCGPDPSLSDELISILNELTQLSKSEHCKVALRARQILIASHLPSYELRHNQVESIFLSAIDMYGHQFCPENLKKLILSETTIFDVLPTFFYHANKVVCMASLEVYVRRGYIAYELNSLQHRQLPDGTCVVEFQFMLPSSHPNRMTVPISITNPDLLRHSTELFMDSGFSPLCQRMGAMVAFRRFEDFTRNFDEVISCFANVPKDTPLFSEARTSLYSEDDCKSLREEPIHILNVSIQCADHLEDEALVPILRTFVQSKKNILVDYGLRRITFLIAQEKEFPKFFTFRARDEFAEDRIYRHLEPALAFQLELNRMRNFDLTAVPCANHKMHLYLGAAKVKEGVEVTDHRFFIRAIIRHSDLITKEASFEYLQNEGERLLLEAMDELEVAFNNTSVRTDCNHIFLNFVPTVIMDPFKIEESVRYMVMRYGSRLWKLRVLQAEVKINIRQTTTGSAVPIRLFITNESGYYLDISLYKEVTDSRSGNIMFHSFGNKQGPQHGMLINTPYVTKDLLQAKRFQAQTLGTTYIYDFPEMFRQALFKLWGSPDKYPKDILTYTELVLDSQGQLVEMNRLPGGNEVGMVAFKMRFKTQEYPEGRDVIVIGNDITFRIGSFGPGEDLLYLRASEMARAEGIPKIYVAANSGARIGMAEEIKHMFHVAWVDPEDPHKGFKYLYLTPQDYTRISSLNSVHCKHIEEGGESRYMITDIIGKDDGLGVENLRGSGMIAGESSLAYEEIVTISLVTCRAIGIGAYLVRLGQRVIQVENSHIILTGASALNKVLGREVYTSNNQLGGVQIMHYNGVSHITVPDDFEGVYTILEWLSYMPKDNHSPVPIITPTDPIDREIEFLPSRAPYDPRWMLAGRPHPTLKGTWQSGFFDHGSFKEIMAPWAQTVVTGRARLGGIPVGVIAVETRTVEVAVPADPANLDSEAKIIQQAGQVWFPDSAYKTAQAVKDFNREKLPLMIFANWRGFSGGMKDMYDQVLKFGAYIVDGLRQYKQPILIYIPPYAELRGGSWVVIDATINPLCIEMYADKESRGGVLEPEGTVEIKFRKKDLIKSMRRIDPAYKKLMEQLGEPDLSDKDRKDLEGRLKAREDLLLPIYHQVAVQFADFHDTPGRMLEKGVISDILEWKTARTFLYWRLRRLLLEDQVKQEILQASGELSHVHIQSMLRRWFVETEGAVKAYLWDNNQVVVQWLEQHWQAGDGPRSTIRENITYLKHDSVLKTIRGLVEENPEVAVDCVIYLSQHISPAERAQVVHLLSTMDSPAST
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion
Function
- Function:
- Catalyzes the ATP-dependent carboxylation of acetyl-CoA to malonyl-CoA. Carries out three functions: biotin carboxyl carrier protein, biotin carboxylase and carboxyltransferase. Involved in inhibition of fatty acid and glucose oxidation and enhancement of fat storage (By similarity). May play a role in regulation of mitochondrial fatty acid oxidation through malonyl-CoA-dependent inhibition of carnitine palmitoyltransferase 1 (By similarity).
- Pathway:
- Lipid metabolism; malonyl-CoA biosynthesis; malonyl-CoA from acetyl-CoA: step 1/1.
- Catalytic Activity:
- ATP + acetyl-CoA + HCO(3)(-) = ADP + phosphate + malonyl-CoA.
- Cofactor:
- COFACTOR: Name=biotin; Xref=ChEBI:CHEBI:57586;
- Kinetics:
- BIOPHYSICOCHEMICAL PROPERTIES: Kinetic parameters: KM=120 uM for ATP
- Enzyme Regulation:
- ENZYME REGULATION: Activity is increased by oligomerization. Activated by citrate. Citrate and MID1IP1 promote oligomerization. Inhibited by malonyl-CoA.
- Active Site:
- ACT_SITE 584 584
- Cross Reference Drug Bank:
- DB00173 DB00121
- Gene Ontology Go:
- cytosol
endomembrane system
mitochondrial outer membrane
mitochondrion
nucleus
acetyl-CoA carboxylase activity
ATP binding
biotin carboxylase activity
metal ion binding
acetyl-CoA metabolic process
biotin metabolic process
carnitine shuttle
cellular lipid metabolic process
energy homeostasis
energy reserve metabolic process
fatty acid biosynthetic process
malonyl-CoA biosynthetic process
negative regulation of catalytic activity
negative regulation of fatty acid beta-oxidation
negative regulation of gene expression
positive regulation of cellular metabolic process
positive regulation of heart growth
positive regulation of lipid storage
protein homotetramerization
regulation of glucose metabolic process
response to nutrient levels
small molecule metabolic process
vitamin metabolic process
water-soluble vitamin metabolic process - Gene Ontology Biological Process:
- acetyl-CoA metabolic process
biotin metabolic process
carnitine shuttle
cellular lipid metabolic process
energy homeostasis
energy reserve metabolic process
fatty acid biosynthetic process
malonyl-CoA biosynthetic process
negative regulation of catalytic activity
negative regulation of fatty acid beta-oxidation
negative regulation of gene expression
positive regulation of cellular metabolic process
positive regulation of heart growth
positive regulation of lipid storage
protein homotetramerization
regulation of glucose metabolic process
response to nutrient levels
small molecule metabolic process
vitamin metabolic process
water-soluble vitamin metabolic process - Gene Ontology Molecular Function:
- acetyl-CoA carboxylase activity
ATP binding
biotin carboxylase activity
metal ion binding - Gene Ontology Cellular Component:
- cytosol
endomembrane system
mitochondrial outer membrane
mitochondrion
nucleus - Keywords:
- 3D-structure
ATP-binding
Allosteric enzyme
Alternative splicing
Biotin
Complete proteome
Fatty acid biosynthesis
Fatty acid metabolism
Ligase
Lipid biosynthesis
Lipid metabolism
Manganese
Membrane
Metal-binding
Mitochondrion
Multifunctional enzyme
Nucleotide-binding
Nucleus
Phosphoprotein
Polymorphism
Reference proteome
Transit peptide - Interacts With:
- P50747