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4-phenylbutyric acid and Bile Duct Obstruction, Intrahepatic

4-phenylbutyric acid has been researched along with Bile Duct Obstruction, Intrahepatic in 13 studies

4-phenylbutyric acid: RN refers to the parent cpd
4-phenylbutyric acid : A monocarboxylic acid the structure of which is that of butyric acid substituted with a phenyl group at C-4. It is a histone deacetylase inhibitor that displays anticancer activity. It inhibits cell proliferation, invasion and migration and induces apoptosis in glioma cells. It also inhibits protein isoprenylation, depletes plasma glutamine, increases production of foetal haemoglobin through transcriptional activation of the gamma-globin gene and affects hPPARgamma activation.

Research Excerpts

ExcerptRelevanceReference
" Discontinuation of rifampin therapy, which had been used to treat pruritus, resulted in reversible severe acute liver injury that was potentially the result of phenylacetate toxicity."3.83Possible Phenylacetate Hepatotoxicity During 4-Phenylbutyrate Therapy of Byler Disease. ( Morris, A; Shneider, BL; Vockley, J, 2016)
" Therapy with 4PB had no beneficial effect for 1 month at 200 mg/kg/day and the next month at 350 mg/kg/day but partially restored BSEP expression at the canalicular membrane and significantly improved liver tests and pruritus at a dosage of 500 mg/kg/day."1.40Improved liver function and relieved pruritus after 4-phenylbutyrate therapy in a patient with progressive familial intrahepatic cholestasis type 2. ( Hayashi, H; Igarashi, K; Inoue, T; Inui, A; Kage, M; Kusuhara, H; Nagai, T; Nagasaka, H; Naoi, S; Sugiyama, Y; Takikawa, H; Tanikawa, K, 2014)
"The therapeutic potency of 4PB in these patients was tested by oral administration of this drug with gradually increasing dosage (200, 350, and 500 mg/kg/day) for 6 months."1.40Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1. ( Bessho, K; Hanada, K; Hasegawa, Y; Hayashi, H; Igarashi, K; Kimura, T; Kondou, H; Konishi, A; Kusuhara, H; Miyoshi, Y; Nagasaka, H; Nakao, K; Naoi, S; Ozono, K, 2014)

Research

Studies (13)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's3 (23.08)29.6817
2010's9 (69.23)24.3611
2020's1 (7.69)2.80

Authors

AuthorsStudies
Misawa, T1
Hayashi, H6
Sugiyama, Y4
Hashimoto, Y1
Nakano, S1
Osaka, S1
Sabu, Y1
Minowa, K1
Hirai, S1
Kondou, H2
Kimura, T2
Azuma, Y1
Watanabe, S1
Inui, A2
Bessho, K2
Nakamura, H1
Kusano, H1
Nakazawa, A1
Tanikawa, K2
Kage, M2
Shimizu, T1
Kusuhara, H3
Zen, Y1
Suzuki, M1
Amzal, R1
Thébaut, A1
Lapalus, M1
Almes, M1
Grosse, B3
Mareux, E1
Collado-Hilly, M1
Davit-Spraul, A3
Bidou, L1
Namy, O1
Jacquemin, E3
Gonzales, E3
Malatack, JJ1
Doyle, D1
Naoi, S2
Inoue, T1
Igarashi, K2
Nagasaka, H2
Takikawa, H1
Nagai, T1
Hasegawa, Y1
Hanada, K1
Nakao, K1
Konishi, A1
Miyoshi, Y1
Ozono, K1
Schuller, B1
Conti, F1
Guettier, C1
Cassio, D2
Shneider, BL1
Morris, A1
Vockley, J1
van der Woerd, WL1
Wichers, CG1
Vestergaard, AL1
Andersen, JP1
Paulusma, CC1
Houwen, RH1
van de Graaf, SF1
Fabre, M1
Lam, P1
Pearson, CL1
Soroka, CJ1
Xu, S1
Mennone, A1
Boyer, JL1

Clinical Trials (1)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Phenylbutyrate for Monogenetic Developmental and Epileptic Encephalopathy[NCT04937062]Early Phase 150 participants (Anticipated)Interventional2021-03-01Enrolling by invitation
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Reviews

1 review available for 4-phenylbutyric acid and Bile Duct Obstruction, Intrahepatic

ArticleYear
A Drug Regimen for Progressive Familial Cholestasis Type 2.
    Pediatrics, 2018, Volume: 141, Issue:1

    Topics: ATP Binding Cassette Transporter, Subfamily B, Member 11; Benzothiepins; Biological Transport; Carba

2018

Other Studies

12 other studies available for 4-phenylbutyric acid and Bile Duct Obstruction, Intrahepatic

ArticleYear
Discovery and structural development of small molecules that enhance transport activity of bile salt export pump mutant associated with progressive familial intrahepatic cholestasis type 2.
    Bioorganic & medicinal chemistry, 2012, May-01, Volume: 20, Issue:9

    Topics: Amino Acid Substitution; Animals; ATP Binding Cassette Transporter, Subfamily B, Member 11; ATP-Bind

2012
Effect of food on the pharmacokinetics and therapeutic efficacy of 4-phenylbutyrate in progressive familial intrahepatic cholestasis.
    Scientific reports, 2019, 11-19, Volume: 9, Issue:1

    Topics: Antineoplastic Agents; ATP Binding Cassette Transporter, Subfamily B, Member 11; Child; Child, Presc

2019
Pharmacological Premature Termination Codon Readthrough of ABCB11 in Bile Salt Export Pump Deficiency: An In Vitro Study.
    Hepatology (Baltimore, Md.), 2021, Volume: 73, Issue:4

    Topics: Animals; ATP Binding Cassette Transporter, Subfamily B, Member 11; Cholestasis, Intrahepatic; Codon,

2021
Improved liver function and relieved pruritus after 4-phenylbutyrate therapy in a patient with progressive familial intrahepatic cholestasis type 2.
    The Journal of pediatrics, 2014, Volume: 164, Issue:5

    Topics: ATP Binding Cassette Transporter, Subfamily B, Member 11; ATP-Binding Cassette Transporters; Cholest

2014
Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1.
    Orphanet journal of rare diseases, 2014, Jul-15, Volume: 9

    Topics: Administration, Oral; Adolescent; Child; Child, Preschool; Cholestasis, Intrahepatic; Disease Progre

2014
Targeted pharmacotherapy in progressive familial intrahepatic cholestasis type 2: Evidence for improvement of cholestasis with 4-phenylbutyrate.
    Hepatology (Baltimore, Md.), 2015, Volume: 62, Issue:2

    Topics: Animals; ATP Binding Cassette Transporter, Subfamily B, Member 11; ATP-Binding Cassette Transporters

2015
Possible Phenylacetate Hepatotoxicity During 4-Phenylbutyrate Therapy of Byler Disease.
    Journal of pediatric gastroenterology and nutrition, 2016, Volume: 62, Issue:3

    Topics: Antineoplastic Agents; Bilirubin; Chemical and Drug Induced Liver Injury; Child, Preschool; Cholesta

2016
Rescue of defective ATP8B1 trafficking by CFTR correctors as a therapeutic strategy for familial intrahepatic cholestasis.
    Journal of hepatology, 2016, Volume: 64, Issue:6

    Topics: Adenosine Triphosphatases; Cell Line, Tumor; Cell Polarity; Cholestasis, Intrahepatic; Cystic Fibros

2016
Short-chain ubiquitination is associated with the degradation rate of a cell-surface-resident bile salt export pump (BSEP/ABCB11).
    Molecular pharmacology, 2009, Volume: 75, Issue:1

    Topics: Adenoviridae; Animals; Antineoplastic Agents; ATP Binding Cassette Transporter, Subfamily B, Member

2009
Successful mutation-specific chaperone therapy with 4-phenylbutyrate in a child with progressive familial intrahepatic cholestasis type 2.
    Journal of hepatology, 2012, Volume: 57, Issue:3

    Topics: Antineoplastic Agents; ATP Binding Cassette Transporter, Subfamily B, Member 11; ATP-Binding Cassett

2012
4-phenylbutyrate enhances the cell surface expression and the transport capacity of wild-type and mutated bile salt export pumps.
    Hepatology (Baltimore, Md.), 2007, Volume: 45, Issue:6

    Topics: Animals; Antineoplastic Agents; ATP Binding Cassette Transporter, Subfamily B, Member 11; ATP-Bindin

2007
Levels of plasma membrane expression in progressive and benign mutations of the bile salt export pump (Bsep/Abcb11) correlate with severity of cholestatic diseases.
    American journal of physiology. Cell physiology, 2007, Volume: 293, Issue:5

    Topics: Animals; ATP Binding Cassette Transporter, Subfamily B, Member 11; ATP-Binding Cassette Transporters

2007