BAAT

The protein encoded by this gene is a liver enzyme that catalyzes the transfer of C24 bile acids from the acyl-CoA thioester to either glycine or taurine, the second step in the formation of bile acid-amino acid conjugates. The bile acid conjugates then act as a detergent in the gastrointestinal tract, which enhances lipid and fat-soluble vitamin absorption. Defects in this gene are a cause of familial hypercholanemia (FHCA). Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq]

Full Name

bile acid Coenzyme A: amino acid N-acyltransferase (glycine N-choloyltransferase)

Function

Catalyzes the amidation of bile acids (BAs) with the amino acids taurine and glycine (PubMed:12810727, PubMed:8034703, PubMed:2037576, PubMed:12239217).
More than 95% of the BAs are N-acyl amidates with glycine and taurine (PubMed:8034703).
Amidation of BAs in the liver with glycine or taurine prior to their excretion into bile is an important biochemical event in bile acid metabolism (PubMed:12810727).
This conjugation (or amidation) plays several important biological roles in that it promotes the secretion of BAs and cholesterol into bile and increases the detergent properties of BAs in the intestine, which facilitates lipid and vitamin absorption (PubMed:12810727).
May also act as an acyl-CoA thioesterase that regulates intracellular levels of free fatty acids (PubMed:12810727, PubMed:8034703, PubMed:12239217).
In vitro, catalyzes the hydrolysis of long- and very long-chain saturated acyl-CoAs to the free fatty acid and coenzyme A (CoASH), and conjugates glycine to these acyl-CoAs (PubMed:12810727).

Biological Process

Acyl-CoA metabolic process Source: UniProtKB
Animal organ regeneration Source: Ensembl
Bile acid biosynthetic process Source: UniProtKB
Bile acid conjugation Source: UniProtKB
Bile acid metabolic process Source: Reactome
Fatty acid metabolic process Source: GO_Central
Glycine metabolic process Source: UniProtKB
Liver development Source: Ensembl
Protein localization Source: Reactome
Taurine metabolic process Source: UniProtKB

Cellular Location

Cytosol; Peroxisome

Involvement in disease

Familial hypercholanemia (FHCA): A disorder characterized by elevated serum bile acid concentrations, itching, and fat malabsorption.