BAAT Background Information BAAT (bile acid coenzyme A (CoA): amino acid N-acyltransferase), also known as BAT, BACAT or glycine N-choloyltransferase, is a member of the C/M/P thioester hydrolase family of proteins. Localizing to the cytoplasm and to peroxisomes, BAAT plays an essential role in bile acid metabolism, being the sole enzyme responsible for catalyzing the second step in the conjugation of bile acids to taurine or glycine. The first step in this reaction is the conversion of bile acids to CoA thioesters by ACSVL6 (bile acid CoA ligase). The conjugation of bile acids is important for its excretion into bile and it is also important for protection against toxicity by the accumulation of unconjugated bile acids. BAAT can be found in liver, pancreas, intestine and gallbladder mucosa. Mutations in the gene encoding BAAT have been associated with familial hypercholanemia (FHCA), a disease characterized by fat malabsorption, an increase in serum bile acid concentrations and itching.
