Short- and medium-chain fatty acids enhance the cell surface expression and transport capacity of the bile salt export pump (BSEP/ABCB11)

4-phenylbutyrate enhances the cell surfa

4-phenylbutyrate enhances the cell surface expression and the transport capacity of wild-type and mutated bile salt export pumps

✍ Hisamitsu Hayashi; Yuichi Sugiyama 📂 Article 📅 2007 🏛 John Wiley and Sons 🌐 English ⚖ 862 KB

Progressive familial intrahepatic cholestasis type 2 (PFIC2) is caused by a mutation in the bile salt export pump (BSEP/ABCB11) gene. We previously reported that E297G and D482G BSEP, which are frequently found mutations in European patients, result in impaired membrane trafficking, whereas both mut