Acetyl-Coa Acetyltransferase (ACAT1) ELISA Kit for Rat

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.

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 2.3.1.9; Lipid Metabolism - fatty acid; Lipid Metabolism - synthesis and degradation of ketone bodies; Mitochondrial; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis

Chromosomal Location of Human Ortholog: 8q24

Cellular Component: mitochondrial inner membrane; mitochondrial matrix; mitochondrion; peroxisome

Molecular Function: acetyl-CoA C-acetyltransferase activity; carbon-carbon lyase activity; coenzyme binding; enzyme binding; ligase activity, forming carbon-carbon bonds; metal ion binding; protein homodimerization activity

Biological Process: acetyl-CoA biosynthetic process; acetyl-CoA catabolic process; brain development; coenzyme A biosynthetic process; coenzyme A metabolic process; fatty acid beta-oxidation; isoleucine catabolic process; ketone body catabolic process; liver development; protein homooligomerization; response to hormone; response to organic cyclic compound; response to starvation