AI Article Synopsis
- The bile salt export pump (BSEP, ABCB11) is essential for removing bile salts from the liver and has significant implications for liver disease progression.
- Two specific mutations in the BSEP gene influence bile acid output and composition, affecting clinical outcomes in children with liver issues.
- A common genetic variation (V444A) in BSEP is linked to better treatment success in chronic hepatitis C cases, possibly due to the role of bile acids in viral replication and immune response.
Article Abstract
The bile salt export pump (BSEP, ABCB11) is the major transporter protein for the excretion of bile salts into bile. Here we describe a spectrum of BSEP-dependent effects on the course of liver diseases, and present two mutations that differentially affect total bile acid output and the biliary bile acid profile. According to the clinical course of affected children, low bile acid output but a normal profile was less harmful than higher output in combination with changes in the ratio of chenodeoxycholic acid to cholic acid. On the other hand, the common BSEP polymorphism V444A (c.1331T>C; allele frequency 65%) emerged as an independent predictor of the success rate in patients with chronic hepatitis C treated with pegylated interferon/ribavirin. This association between bile acid transport and hepatitis C may be due to interference of bile acids with viral replication, interferon signaling or antiviral proteins.
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| http://dx.doi.org/10.1159/000324140 | DOI Listing |
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