Targeted pharmacotherapy in progressive familial intrahepatic cholestasis type 2: Evidence for improvement of cholestasis with 4-phenylbutyrate
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Title
Targeted pharmacotherapy in progressive familial intrahepatic cholestasis type 2: Evidence for improvement of cholestasis with 4-phenylbutyrate
Authors
Keywords
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Journal
HEPATOLOGY
Volume 62, Issue 2, Pages 558-566
Publisher
Wiley
Online
2015-02-26
DOI
10.1002/hep.27767
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Related references
Note: Only part of the references are listed.- Improved Liver Function and Relieved Pruritus after 4-Phenylbutyrate Therapy in a Patient with Progressive Familial Intrahepatic Cholestasis Type 2
- (2014) Sotaro Naoi et al. JOURNAL OF PEDIATRICS
- Liver transcript analysis reveals aberrant splicing due to silent and intronic variations in the ABCB11 gene
- (2014) Anne Davit-Spraul et al. MOLECULAR GENETICS AND METABOLISM
- Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1
- (2014) Yasuhiro Hasegawa et al. Orphanet Journal of Rare Diseases
- Successful mutation-specific chaperone therapy with 4-phenylbutyrate in a child with progressive familial intrahepatic cholestasis type 2
- (2012) Emmanuel Gonzales et al. JOURNAL OF HEPATOLOGY
- ATP8B1 and ABCB11 analysis in 62 children with normal gamma-glutamyl transferase progressive familial intrahepatic cholestasis (PFIC): Phenotypic differences between PFIC1 and PFIC2 and natural history
- (2010) Anne Davit-Spraul et al. HEPATOLOGY
- Mutation specific drug therapy for progressive familial or benign recurrent intrahepatic cholestasis: A new tool in a near future?
- (2010) Emmanuel Gonzales et al. JOURNAL OF HEPATOLOGY
- Favorable effect of 4-phenylacetate on liver functions attributable to enhanced bile salt export pump expression in ornithine transcarbamylase-deficient children
- (2010) Hironori Nagasaka et al. MOLECULAR GENETICS AND METABOLISM
- Folding defects in P-type ATP 8B1 associated with hereditary cholestasis are ameliorated by 4-phenylbutyrate
- (2009) Lieke M. van der Velden et al. HEPATOLOGY
- Reduced expression of ATP7B affected by Wilson disease-causing mutations is rescued by pharmacological folding chaperones 4-phenylbutyrate and curcumin
- (2009) Peter V. E. van den Berghe et al. HEPATOLOGY
- Liver disease associated with canalicular transport defects: Current and future therapies
- (2009) Janneke M. Stapelbroek et al. JOURNAL OF HEPATOLOGY
- Severe Bile Salt Export Pump Deficiency: 82 Different ABCB11 Mutations in 109 Families
- (2008) Sandra S. Strautnieks et al. GASTROENTEROLOGY
- Missense mutations and single nucleotide polymorphisms in ABCB11 impair bile salt export pump processing and function or disrupt pre-messenger RNA splicing
- (2008) Jane A. Byrne et al. HEPATOLOGY
- A missense mutation in ABCB4 gene involved in progressive familial intrahepatic cholestasis type 3 leads to a folding defect that can be rescued by low temperature
- (2008) Jean-Louis Delaunay et al. HEPATOLOGY
- Degradation of the bile salt export pump at endoplasmic reticulum in progressive familial intrahepatic cholestasis type II
- (2008) Lin Wang et al. HEPATOLOGY
- Phenotypic differences in PFIC2 and BRIC2 correlate with protein stability of mutant Bsep and impaired taurocholate secretion in MDCK II cells
- (2007) Tatehiro Kagawa et al. AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY
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