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

Pub. Date : 2007 Jun

PMID : 17538928






4 Functional Relationships(s)
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1 Because sodium 4-phenylbutyrate (4PBA) has been shown to restore the reduced cell surface expression of mutated plasma membrane proteins, in the current study, we investigated the effect of 4PBA treatment on E297G and D482G BSEP. 4-phenylbutyric acid ATP binding cassette subfamily B member 11 Homo sapiens
2 Transcellular transport and cell surface biotinylation studies using Madin-Darby canine kidney (MDCK) II cells demonstrated that 4PBA treatment increased functional cell surface expression of wild-type (WT), E297G, and D482G BSEP. 4-phenylbutyric acid ATP binding cassette subfamily B member 11 Homo sapiens
3 4PBA is a potential pharmacological agent for treating not only PFIC2 patients with E297G and D482G mutations but also other cholestatic patients, in whom the BSEP expression at the canalicular membrane is reduced. 4-phenylbutyric acid ATP binding cassette subfamily B member 11 Homo sapiens
4 4PBA is a potential pharmacological agent for treating not only PFIC2 patients with E297G and D482G mutations but also other cholestatic patients, in whom the BSEP expression at the canalicular membrane is reduced. 4-phenylbutyric acid ATP binding cassette subfamily B member 11 Homo sapiens