ACAT1 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. 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. The activity of the enzyme is reversible and it can also catalyze the condensation of two acetyl-CoA molecules into acetoacetyl-CoA. Thereby, it plays a major role in ketone body metabolism. Belongs to the thiolase-like superfamily. Thiolase family. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: Acetyltransferase; Amino Acid Metabolism - lysine degradation; Amino Acid Metabolism - tryptophan; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - butanoate; Carbohydrate Metabolism - propanoate; Carbohydrate Metabolism - pyruvate; EC; Lipid Metabolism - fatty acid; Lipid Metabolism - synthesis and degradation of ketone bodies; Mitochondrial; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis
Chromosomal Location of human Ortholog: 11q22.3
Cellular Component:  endoplasmic reticulum; mitochondrial matrix; mitochondrion
Molecular Function:  acetyl-CoA C-acetyltransferase activity; C-acetyltransferase activity; cholesterol O-acyltransferase activity; coenzyme A binding; enzyme binding; identical protein binding; potassium ion binding
Biological Process:  acetyl-CoA biosynthetic process; acetyl-CoA catabolic process; adipose tissue development; coenzyme A biosynthetic process; coenzyme A metabolic process; fatty acid beta-oxidation; isoleucine catabolic process; ketone body catabolic process; ketone body metabolic process; liver development; metanephric proximal convoluted tubule development; propionyl-CoA biosynthetic process; response to hormone; response to organic cyclic compound; response to starvation
Disease: Alpha-methylacetoacetic Aciduria
Reference #:  P24752 (UniProtKB)
Alt. Names/Synonyms: ACAT; ACAT1; acetoacetyl Coenzyme A thiolase; Acetoacetyl-CoA thiolase; acetyl-CoA acetyltransferase 1; Acetyl-CoA acetyltransferase, mitochondrial; acetyl-Coenzyme A acetyltransferase 1; MAT; mitochondrial acetoacetyl-CoA thiolase; T2; testicular tissue protein Li 198; THIL
Gene Symbols: ACAT1
Molecular weight: 45,200 Da
Basal Isoelectric point: 8.98  Predict pI for various phosphorylation states
Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology® Total Proteins
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Protein Structure Not Found.

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