PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 27757140-3 2016 Sodium 4-phenylbutyrate (4-PB) seems to be a potential therapeutic compound for PFIC2. 4-phenylbutyric acid 0-23 ATP binding cassette subfamily B member 11 Homo sapiens 80-85 29284646-6 2018 We report a multidrug regimen of 4-phenylbutyrate, oxcarbazepine, and maralixibat (an experimental drug owned by Shire Pharmaceuticals, Dublin, Republic of Ireland) that completely controlled symptoms in 2 siblings with partial loss of BSEP activity. 4-phenylbutyric acid 33-49 ATP binding cassette subfamily B member 11 Homo sapiens 236-240 27757140-3 2016 Sodium 4-phenylbutyrate (4-PB) seems to be a potential therapeutic compound for PFIC2. 4-phenylbutyric acid 25-29 ATP binding cassette subfamily B member 11 Homo sapiens 80-85 17538928-9 2007 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 0-4 ATP binding cassette subfamily B member 11 Homo sapiens 64-69 25716872-4 2015 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 120-124 ATP binding cassette subfamily B member 11 Homo sapiens 5-10 25716872-8 2015 Treatment with 4-PB and UDCA partially corrected Bsep mutant targeting. 4-phenylbutyric acid 15-19 ATP binding cassette subfamily B member 11 Homo sapiens 49-53 25716872-13 2015 CONCLUSION: 4-PB therapy may be efficient in selected patients with PFIC2 owing to ABCB11 missense mutations affecting BSEP canalicular targeting. 4-phenylbutyric acid 12-16 ATP binding cassette subfamily B member 11 Homo sapiens 68-73 25716872-13 2015 CONCLUSION: 4-PB therapy may be efficient in selected patients with PFIC2 owing to ABCB11 missense mutations affecting BSEP canalicular targeting. 4-phenylbutyric acid 12-16 ATP binding cassette subfamily B member 11 Homo sapiens 83-89 25716872-13 2015 CONCLUSION: 4-PB therapy may be efficient in selected patients with PFIC2 owing to ABCB11 missense mutations affecting BSEP canalicular targeting. 4-phenylbutyric acid 12-16 ATP binding cassette subfamily B member 11 Homo sapiens 119-123 25716872-14 2015 Bile secretion improvement may be a result of the ability of 4-PB to retarget mutated BSEP. 4-phenylbutyric acid 61-65 ATP binding cassette subfamily B member 11 Homo sapiens 86-90 22262466-4 2012 We have shown previously that 4-phenylbutyrate (4PBA), a drug used for ornithine transcarbamylase deficiency (OTCD), inhibited internalization and subsequent degradation of cell-surface-resident BSEP. 4-phenylbutyric acid 30-46 ATP binding cassette subfamily B member 11 Homo sapiens 195-199 26223708-0 2016 Successful treatment with 4-phenylbutyrate in a patient with benign recurrent intrahepatic cholestasis type 2 refractory to biliary drainage and bilirubin absorption. 4-phenylbutyric acid 26-42 ATP binding cassette subfamily B member 11 Homo sapiens 61-109 26223708-3 2016 We previously provided experimental and clinical evidence suggesting the therapeutic potential of 4-phenylbutyrate (4PB) for the cholestatic attacks of BRIC2. 4-phenylbutyric acid 98-114 ATP binding cassette subfamily B member 11 Homo sapiens 152-157 25022842-4 2014 We found a previously unidentified pharmacological effect of 4-phenylbutyrate (4PB) that increases the expression and function of BSEP. 4-phenylbutyric acid 61-77 ATP binding cassette subfamily B member 11 Homo sapiens 130-134 25022842-4 2014 We found a previously unidentified pharmacological effect of 4-phenylbutyrate (4PB) that increases the expression and function of BSEP. 4-phenylbutyric acid 79-82 ATP binding cassette subfamily B member 11 Homo sapiens 130-134 25274209-5 2014 We have previously shown that in many cases of PFIC2 patients, the dysfunction of BSEP is attributable to decreased BSEP expression on the hepatocanalicular membrane and that 4-phenylbutyrate (4PB), an approved drug for urea cycle disorder, may be a compound with potential to restore BSEP expression. 4-phenylbutyric acid 175-191 ATP binding cassette subfamily B member 11 Homo sapiens 47-52 25274209-5 2014 We have previously shown that in many cases of PFIC2 patients, the dysfunction of BSEP is attributable to decreased BSEP expression on the hepatocanalicular membrane and that 4-phenylbutyrate (4PB), an approved drug for urea cycle disorder, may be a compound with potential to restore BSEP expression. 4-phenylbutyric acid 175-191 ATP binding cassette subfamily B member 11 Homo sapiens 82-86 22609309-4 2012 After an in vitro study in a hepatocellular polarized line, we tested 4-PB treatment in a child with a homozygous p.T1210P BSEP mutation. 4-phenylbutyric acid 70-74 ATP binding cassette subfamily B member 11 Homo sapiens 123-127 22609309-9 2012 4-PB as well as incubation at 27 C partially corrected Bsep(T1210P)-GFP targeting to the canalicular membrane, while combined treatments resulted in normal canalicular localization. 4-phenylbutyric acid 0-4 ATP binding cassette subfamily B member 11 Homo sapiens 66-71 22609309-12 2012 CONCLUSIONS: The results illustrate for the first time the therapeutic potential of a clinically approved chaperone drug in a selected patient with PFIC2 and support that bile secretion improvement might be due to the ability of 4-PB to retarget mutated BSEP. 4-phenylbutyric acid 229-233 ATP binding cassette subfamily B member 11 Homo sapiens 148-153 22609309-12 2012 CONCLUSIONS: The results illustrate for the first time the therapeutic potential of a clinically approved chaperone drug in a selected patient with PFIC2 and support that bile secretion improvement might be due to the ability of 4-PB to retarget mutated BSEP. 4-phenylbutyric acid 229-233 ATP binding cassette subfamily B member 11 Homo sapiens 254-258 22464344-2 2012 4-Phenylbutyric acid (4-PBA) enhances the cell surface expression and transport capacity of E297G BSEP, but has a relatively high dose (1mM or more) is required to show the effect. 4-phenylbutyric acid 0-20 ATP binding cassette subfamily B member 11 Homo sapiens 98-102 22464344-2 2012 4-Phenylbutyric acid (4-PBA) enhances the cell surface expression and transport capacity of E297G BSEP, but has a relatively high dose (1mM or more) is required to show the effect. 4-phenylbutyric acid 22-27 ATP binding cassette subfamily B member 11 Homo sapiens 98-102 17538928-4 2007 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 190-194 ATP binding cassette subfamily B member 11 Homo sapiens 224-228 17538928-5 2007 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 129-133 ATP binding cassette subfamily B member 11 Homo sapiens 225-229 17538928-9 2007 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 0-4 ATP binding cassette subfamily B member 11 Homo sapiens 159-163