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Details on Person UniProt:P24752 ACAT1
| Class:Id | ReferenceGeneProduct:65997 |
|---|---|
| _chainChangeLog | transit peptide:1-33 added on Sat February 7 2015;chain:34-427 added on Sat February 7 2015 |
| _displayName | UniProt:P24752 ACAT1 |
| _timestamp | 2025-08-15 21:57:38 |
| chain | transit peptide:1-33 chain:34-427 |
| checksum | 2E81168EB39D0142 |
| comment | FUNCTION This is one of the enzymes that catalyzes the last step of the mitochondrial beta-oxidation pathway, an aerobic process breaking down fatty acids into acetyl-CoA (PubMed:1715688, PubMed:7728148, PubMed:9744475). Using free coenzyme A/CoA, catalyzes the thiolytic cleavage of medium- to long-chain 3-oxoacyl-CoAs into acetyl-CoA and a fatty acyl-CoA shortened by two carbon atoms (PubMed:1715688, PubMed:7728148, PubMed:9744475). The activity of the enzyme is reversible and it can also catalyze the condensation of two acetyl-CoA molecules into acetoacetyl-CoA (PubMed:17371050). Thereby, it plays a major role in ketone body metabolism (PubMed:1715688, PubMed:17371050, PubMed:7728148, PubMed:9744475).CATALYTIC ACTIVITY 2 acetyl-CoA = acetoacetyl-CoA + CoACATALYTIC ACTIVITY propanoyl-CoA + acetyl-CoA = 2-methyl-3-oxobutanoyl-CoA + CoAACTIVITY REGULATION Activated by potassium ions, but not sodium ions.BIOPHYSICOCHEMICAL PROPERTIES kcat is 21 sec(-1) for the degradation of acetoacetyl-CoA (at 25 degrees Celsius in the presence of 40 mM KCl) (PubMed:17371050). kcat is 8 sec(-1) for the degradation of acetoacetyl-CoA (at 25 degrees Celsius in the presence of 40 mM NaCl) (PubMed:17371050). kcat is 61 sec(-1) for the degradation of 2-methylacetoacetyl-CoA (at 25 degrees Celsius in the presence of 40 mM KCl) (PubMed:17371050). kcat is 14 sec(-1) for the degradation of 2-methylacetoacetyl-CoA (at 25 degrees Celsius in the presence of 40 mM NaCl) (PubMed:17371050). kcat is 3.5 sec(-1) for the synthesis of acetoacetyl-CoA (at 25 degrees Celsius in the presence of 40 mM KCl) (PubMed:17371050).PATHWAY Lipid metabolism; fatty acid beta-oxidation.SUBUNIT Homotetramer.SUBCELLULAR LOCATION Succinylation at Lys-268, adjacent to a coenzyme A binding site. Desuccinylated by SIRT5 (By similarity).DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the thiolase-like superfamily. Thiolase family. |
| description | recommendedName: Acetyl-CoA acetyltransferase, mitochondrial ecNumber evidence="4 7 8 10 12"2.3.1.9 alternativeName: Acetoacetyl-CoA thiolase alternativeName: T2 |
| geneName | ACAT1 ACAT MAT |
| identifier | P24752 |
| isSequenceChanged | FALSE |
| keyword | 3D-structure Acetylation Acyltransferase Alternative splicing Direct protein sequencing Disease variant Fatty acid metabolism Lipid metabolism Metal-binding Mitochondrion Potassium Proteomics identification Reference proteome Transferase Transit peptide |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9917590] Weiser, Joel, 2024-08-09 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 [InstanceEdit:9963647] Weiser, Joel, 2025-08-15 |
| name | ACAT1 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8957956] ENSEMBL:ENSG00000075239 ACAT1 [Homo sapiens] |
| secondaryIdentifier | THIL_HUMAN B2R6H1 G3XAB4 Q96FG8 |
| sequenceLength | 427 |
| species | [Species:48887] Homo sapiens |
| (isoformParent) | [ReferenceIsoform:8966321] UniProt:P24752-1 ACAT1 [Homo sapiens] [ReferenceIsoform:8966322] UniProt:P24752-2 ACAT1 [Homo sapiens] |
| (referenceEntity) | [EntityWithAccessionedSequence:70838] ACAT1(35-427) [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:8958528] ACAT1 [extracellular region] [Homo sapiens] [EntityWithAccessionedSequence:9915326] N93S ACAT1(35-427) [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9915331] G183R ACAT1(35-427) [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9915332] N158D ACAT1(35-427) [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9915339] A301P ACAT1(35-427) [mitochondrial matrix] [Homo sapiens] [EntityWithAccessionedSequence:9915341] A333P ACAT1(35-427) [mitochondrial matrix] [Homo sapiens] |
| (referenceSequence) | [ReplacedResidue:9915330] glycine 183 replaced with L-arginine [ReplacedResidue:9915335] L-alanine 301 replaced with L-proline [ReplacedResidue:9915336] L-alanine 333 replaced with L-proline [ReplacedResidue:9915338] L-asparagine 158 replaced with L-aspartic acid [ReplacedResidue:9915342] L-asparagine 93 replaced with L-serine |
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No pathways have been reviewed or authored by UniProt:P24752 ACAT1 (65997)
