NP_001121082.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
46,299 Da
NCBI Official Full Name
bile acid-CoA:amino acid N-acyltransferase
NCBI Official Synonym Full Names
bile acid-CoA:amino acid N-acyltransferase
NCBI Official Synonym Symbols
NCBI Protein Information
bile acid-CoA:amino acid N-acyltransferase
UniProt Protein Name
Bile acid-CoA:amino acid N-acyltransferase
UniProt Synonym Protein Names
Glycine N-choloyltransferase; Long-chain fatty-acyl-CoA hydrolase
UniProt Synonym Gene Names
NCBI Summary for 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, Jul 2008]
UniProt Comments for BAAT
Involved in bile acid metabolism. In liver hepatocytes catalyzes the second step in the conjugation of C24 bile acids (choloneates) to glycine and taurine before excretion into bile canaliculi. The major components of bile are cholic acid and chenodeoxycholic acid. In a first step the bile acids are converted to an acyl-CoA thioester, either in peroxisomes (primary bile acids deriving from the cholesterol pathway), or cytoplasmic at the endoplasmic reticulum (secondary bile acids). May catalyze the conjugation of primary or secondary bile acids, or both. The conjugation increases the detergent properties of bile acids in the intestine, which facilitates lipid and fat-soluble vitamin absorption. In turn, bile acids are deconjugated by bacteria in the intestine and are recycled back to the liver for reconjugation (secondary bile acids). May also act as an acyl-CoA thioesterase that regulates intracellular levels of free fatty acids. 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.
Research Articles on BAAT
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Products associated with BAAT recombinant protein
Pathways associated with BAAT recombinant protein
Diseases associated with BAAT recombinant protein
Organs/Tissues associated with BAAT recombinant protein
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