Title : Targeted pharmacotherapy in progressive familial intrahepatic cholestasis type 2: Evidence for improvement of cholestasis with 4-phenylbutyrate.

Pub. Date : 2015 Aug

PMID : 25716872






6 Functional Relationships(s)
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1 Four PFIC2 patients harboring at least one missense mutation (p.G982R, p.R1128C, and p.T1210P) were treated orally with 4-PB and followed prospectively. 4-phenylbutyric acid ATP binding cassette subfamily B member 11 Homo sapiens
2 Treatment with 4-PB and UDCA partially corrected Bsep mutant targeting. 4-phenylbutyric acid ATP binding cassette subfamily B member 11 Homo sapiens
3 CONCLUSION: 4-PB therapy may be efficient in selected patients with PFIC2 owing to ABCB11 missense mutations affecting BSEP canalicular targeting. 4-phenylbutyric acid ATP binding cassette subfamily B member 11 Homo sapiens
4 CONCLUSION: 4-PB therapy may be efficient in selected patients with PFIC2 owing to ABCB11 missense mutations affecting BSEP canalicular targeting. 4-phenylbutyric acid ATP binding cassette subfamily B member 11 Homo sapiens
5 CONCLUSION: 4-PB therapy may be efficient in selected patients with PFIC2 owing to ABCB11 missense mutations affecting BSEP canalicular targeting. 4-phenylbutyric acid ATP binding cassette subfamily B member 11 Homo sapiens
6 Bile secretion improvement may be a result of the ability of 4-PB to retarget mutated BSEP. 4-phenylbutyric acid ATP binding cassette subfamily B member 11 Homo sapiens