BAAT blocking peptide
BAAT Blocking Peptide (N-Term)
NCBI and Uniprot Product Information
NCBI Description
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 Description
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. Defects in BAAT are involved in familial hypercholanemia (FHCA). FHCA is a disorder characterized by elevated serum bile acid concentrations, itching, and fat malabsorption. Belongs to the C/M/P thioester hydrolase family.
Protein type: Hydrolase; Other Amino Acids Metabolism - taurine and hypotaurine; Lipid Metabolism - unsaturated fatty acid biosynthesis; Lipid Metabolism - primary bile acid biosynthesis; EC 2.3.1.65; Transferase; EC 3.1.2.2
Chromosomal Location of Human Ortholog: 9q22.3
Cellular Component: cytosol; peroxisomal matrix; peroxisome
Molecular Function: glycine N-choloyltransferase activity; N-acyltransferase activity; protein binding; receptor binding; transferase activity, transferring acyl groups
Biological Process: acyl-CoA metabolic process; bile acid biosynthetic process; bile acid metabolic process; glycine metabolic process; taurine metabolic process
Disease: Hypercholanemia, Familial
Research Articles on BAAT
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Product Notes
The BAAT baat (Catalog #AAA9230627) is a Blocking Peptide and is intended for research purposes only. The product is available for immediate purchase. It is sometimes possible for the material contained within the vial of "BAAT, Blocking Peptide" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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