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Bile acid malabsorption (BAM), known also as bile acid diarrhea, is a cause of several gut-related problems, the main one being chronic diarrhea.It has also been called bile acid-induced diarrhea, cholerheic or choleretic enteropathy, bile salt diarrhea or bile salt malabsorption.
The product of the ABCB11 gene is an ABC transporter named BSEP (bile salt export pump), or sPgp (sister of P-glycoprotein). This membrane-associated protein is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes.
These conjugated bile acids are often referred to as bile salts. The pKa of the unconjugated bile acids are between 5 and 6.5, [ 4 ] and the pH of the duodenum ranges between 3 and 5, so when unconjugated bile acids are in the duodenum, they are almost always protonated (HA form), which makes them relatively insoluble in water.
Chronic diarrhea may be caused by excess bile salts entering the colon rather than being absorbed at the end of the small intestine (the ileum). This condition of bile acid malabsorption occurs after surgery to the ileum , in Crohn's disease , with a number of other gastrointestinal causes, or is commonly a primary, idiopathic condition.
In the human liver, bile is composed of 97–98% water, 0.7% bile salts, 0.2% bilirubin, 0.51% fats (cholesterol, fatty acids, and lecithin), and 200 meq/L inorganic salts. [ 2 ] [ 3 ] The two main pigments of bile are bilirubin , which is orange-yellow, and its oxidised form biliverdin , which is green.
When this section is removed, the bile acids pass into the large bowel and cause diarrhea due to stimulation of chloride/fluid secretion by the colonocytes resulting in a secretory diarrhea. Colestyramine prevents this increase in water by making the bile acids insoluble and osmotically inactive.
Ursodeoxycholic acid (UDCA), also known as ursodiol, is a secondary bile acid, produced in humans and most other species from metabolism by intestinal bacteria.It is synthesized in the liver in some species, and was first identified in bile of bears of genus Ursus, from which its name derived. [8]
Retention of bile salts within hepatocytes, which are the only cell type to express BSEP, causes hepatocellular damage and cholestasis. [citation needed] PFIC-3 is caused by a variety of mutations in ABCB4, the gene encoding multidrug resistance protein 3 (MDR3), [5] which codes for a floppase responsible for phosphatidylcholine translocation ...
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