ACAT1
ACAT1 (acetyl-CoA acetyltransferase 1, also known as THIL or ACAT) is a mitochondrial thiolase that acts in the final step of fatty acid beta-oxidation and in ketone body metabolism. It catalyses the reversible conversion between two acetyl-CoA molecules and acetoacetyl-CoA. Browse Assay Genie's current range of ACAT1 research reagents below, or read on for the biology behind the target.
ACAT1 is one of the enzymes that catalyses the last step of the mitochondrial beta-oxidation pathway, an aerobic process that breaks fatty acids down into acetyl-CoA. Using free coenzyme A, it carries out 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 reaction is reversible. ACAT1 can also condense two molecules of acetyl-CoA into acetoacetyl-CoA, and in this way it plays a major role in ketone body metabolism. The enzyme is localised to the mitochondrion, and the ACAT1 gene encodes the mitochondrial form of acetyl-CoA acetyltransferase. Mouse and rat orthologues are both designated Acat1.
ACAT1 belongs to the thiolase family within the thiolase-like superfamily, and HGNC places it in the acetyl-CoA acetyltransferases gene group, which also includes ACAT2. The protein has previously been named acetyl-Coenzyme A acetyltransferase 1, and THIL and ACAT are alternative symbols. Defects in the gene are associated with 3-ketothiolase deficiency, an inborn error of isoleucine catabolism. It is characterised by urinary excretion of 2-methyl-3-hydroxybutyric acid, 2-methylacetoacetic acid, tiglylglycine and butanone.
ACAT1 catalyses the last step of mitochondrial beta-oxidation, releasing acetyl-CoA from medium- to long-chain 3-oxoacyl-CoAs.
By condensing two acetyl-CoA molecules into acetoacetyl-CoA, ACAT1 plays a major role in ketone body metabolism.
Defects in ACAT1 cause 3-ketothiolase deficiency, an inborn error of isoleucine catabolism with a distinctive urinary profile.
As a reversible mitochondrial thiolase, ACAT1 is a model for studying acetyl-CoA flux and thiolase family function.
ACAT1 is a mitochondrial thiolase that catalyses the last step of beta-oxidation, cleaving 3-oxoacyl-CoAs into acetyl-CoA and a shortened fatty acyl-CoA. Because the reaction is reversible, it also condenses two acetyl-CoA molecules into acetoacetyl-CoA, which underlies its role in ketone body metabolism.
The HGNC name is acetyl-CoA acetyltransferase 1, previously acetyl-Coenzyme A acetyltransferase 1. UniProt lists it as acetyl-CoA acetyltransferase, mitochondrial. Aliases include THIL and ACAT. Its EC number is 2.3.1.9, and ACAT2 is a related member of the same gene group.
Defects in ACAT1 are associated with 3-ketothiolase deficiency, an inborn error of isoleucine catabolism. Orthologues exist in mouse and rat, both named Acat1, so rodent models can be used to study it. Check the datasheet of any product for its validated reactivity.
Other acetyl-CoA acetyltransferases and related enzymes of fatty acid and ketone body metabolism.
Gene and protein annotations from HGNC, NCBI Gene and UniProt P24752; product specifications from Assay Genie datasheets.