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Acetyl-CoA acetyltransferase (ACAT1)

UniProt Number: P24752
Alternate Names: Acetoacetyl-CoA thiolase, T2, THIL, ACAT, MAT
Structure and Function: Acetyl-Coenzyme A acetyltransferase (ACAT1), also called 2-methylacetoacetyl,AiCoA thiolase or beta-ketothiolase is a mitochondrial enzyme involved in both amino acid degradation and in fatty acid oxidation. It catalyzes the last step in breaking down isoleucine by converting 2-methyl-acetoacetyl-CoA into propionyl-CoA and acetyl CoA. During ketolysis, it converts a acetoacetyl-CoA to two molecules of acetyl CoA. Note that there is a cytosolic form of the enzyme (ACAT2) which can link two molecules of acetyl,AiCoA to acetoacetyl-CoA as an early step in ketogenesis and in fatty acid biogenesis.
Disease Associations: Defects in ACAT1 are a cause of 3-ketothiolase deficiency (3KTD) [MIM:203750], also known as alpha-methylacetoaceticaciduria. 3KTD is an inborn error of isoleucine catabolism characterized by intermittent ketoacidotic attacks associated with unconsciousness.

Monoclonal Antibodies
Cat. No. Name Reactivity Apps. Amount
MS718 ACAT1 antibody human ICC, IHC, IP, ICE, FLOW 100 µg

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Acetyl-CoA Metabolism: Key Nodes


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