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ACAT1 Plays a major role in ketone body metabolism. Defects in ACAT1 are a cause of 3-ketothiolase deficiency (3KTD); also known as alpha- methylacetoaceticaciduria. 3KTD is an inborn error of isoleucine catabolism characterized by intermittent ketoacidotic attacks associated with unconsciousness. Some patients die during an attack or are mentally retarded. Urinary excretion of 2-methyl-3- hydroxybutyric acid, 2-methylacetoacetic acid, triglylglycine, butanone is increased. It seems likely that the severity of this disease correlates better with the environmental or acquired factors than with the ACAT1 genotype. Belongs to the thiolase family. 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: mitochondrial matrix; mitochondrion
Molecular Function: acetyl-CoA C-acetyltransferase activity; carbon-carbon lyase activity; ligase activity, forming carbon-carbon bonds
Biological Process: acetyl-CoA biosynthetic process; acetyl-CoA catabolic process; branched chain family amino acid catabolic process; coenzyme A biosynthetic process; coenzyme A metabolic process; fatty acid beta-oxidation; isoleucine catabolic process; ketone body biosynthetic process; ketone body catabolic process
Disease: Alpha-methylacetoacetic Aciduria
Reference #:  P24752 (UniProtKB)
Alt. Names/Synonyms: ACAT; ACAT1; acetoacetyl Coenzyme A thiolase; Acetoacetyl-CoA thiolase; Acetyl-CoA acetyltransferase, mitochondrial; acetyl-Coenzyme A acetyltransferase 1; MAT; mitochondrial acetoacetyl-CoA thiolase; T2; THIL
Gene Symbols: ACAT1
Molecular weight: 45,200 Da
Basal Isoelectric point: 8.98  Predict pI for various phosphorylation states
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Protein Structure Not Found.
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