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Rabbit Anti-BAAT/FITC Conjugated antibody
background:
BAAT (bile acid Coenzyme A: amino acid N-acyltransferase) is involved in bile acid metabolism. In liver hepatocytes BAAT catalyzes the transfer of the bile acid moiety from the acyl-CoA thioester to glycine and taurine, before excretion into bile canaliculi. This is the second step in the formation of bile acid-amino acid conjugates. The conjugation increases the detergent properties of bile acids in the intestine, which facilitates lipid and fat-soluble vitamin absorption. BAAT may also act as an acyl-CoA thioesterase that regulates intracellular levels of free fatty acids.
Function:
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.
Subcellular Location:
Cytoplasm.
Tissue Specificity:
Expressed in liver, gallbladder mucosa and pancreas.
DISEASE:
Defects in BAAT are involved in familial hypercholanemia (FHCA) [MIM:607748]. FHCA is a disorder characterized by elevated serum bile acid concentrations, itching, and fat malabsorption.
Similarity:
Belongs to the C/M/P thioester hydrolase family.
Database links:
Entrez Gene: 570 Human
Entrez Gene: 12012 Mouse
Entrez Gene: 29725 Rat
Omim: 602938 Human
SwissProt: Q14032 Human
SwissProt: Q91X34 Mouse
SwissProt: Q63276 Rat
Unigene: 284712 Human
Unigene: 2859 Mouse
Unigene: 11129 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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