(Q50438744)

English

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

scientific article published on 26 February 2015

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scholarly article

1 reference

Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

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

1 reference

Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

progressive familial intrahepatic cholestasis

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pharmacotherapy

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Catherine Guettier

6

object named as

Catherine Guettier

1 reference

Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

Emmanuel Jacquemin

8

object named as

Emmanuel Jacquemin

1 reference

Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

author name string

Emmanuel Gonzales

1

1 reference

Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

Brigitte Grosse

2

1 reference

Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

Brice Schuller

3

1 reference

Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

Anne Davit-Spraul

4

1 reference

Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

Filomena Conti

5

1 reference

Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

Doris Cassio

7

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Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

language of work or name

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publication date

26 February 2015

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Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

1 reference

Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

62

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Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

2

1 reference

Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

558-566

1 reference

Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families.

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

A progressive familial intrahepatic cholestasis type 2 mutation causes an unstable, temperature-sensitive bile salt export pump.

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Levels of plasma membrane expression in progressive and benign mutations of the bile salt export pump (Bsep/Abcb11) correlate with severity of cholestatic diseases

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Phenotypic differences in PFIC2 and BRIC2 correlate with protein stability of mutant Bsep and impaired taurocholate secretion in MDCK II cells

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Degradation of the bile salt export pump at endoplasmic reticulum in progressive familial intrahepatic cholestasis type II

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Missense mutations and single nucleotide polymorphisms in ABCB11 impair bile salt export pump processing and function or disrupt pre-messenger RNA splicing

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Long-term treatment with sodium phenylbutyrate in ornithine transcarbamylase-deficient patients

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Folding defects in P-type ATP 8B1 associated with hereditary cholestasis are ameliorated by 4-phenylbutyrate.

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Reduced expression of ATP7B affected by Wilson disease-causing mutations is rescued by pharmacological folding chaperones 4-phenylbutyrate and curcumin

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Mutation specific drug therapy for progressive familial or benign recurrent intrahepatic cholestasis: a new tool in a near future?

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Successful mutation-specific chaperone therapy with 4-phenylbutyrate in a child with progressive familial intrahepatic cholestasis type 2.

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Liver disease associated with canalicular transport defects: current and future therapies

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

How to induce non-polarized cells of hepatic origin to express typical hepatocyte polarity: generation of new highly polarized cell models with developed and functional bile canaliculi

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

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.

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Mechanisms of action and therapeutic efficacy of ursodeoxycholic acid in cholestatic liver disease

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Chemical chaperones reduce ER stress and restore glucose homeostasis in a mouse model of type 2 diabetes

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Effect of Ursodeoxycholic Acid on the Expression of the Hepatocellular Bile Acid Transporters (Ntcp and bsep) in Rats With Estrogen-Induced Cholestasis

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Differential roles of ubiquitination in the degradation mechanism of cell surface-resident bile salt export pump and multidrug resistance-associated protein 2.

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Sequence analysis of bile salt export pump (ABCB11) and multidrug resistance p-glycoprotein 3 (ABCB4, MDR3) in patients with intrahepatic cholestasis of pregnancy

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Favorable effect of 4-phenylacetate on liver functions attributable to enhanced bile salt export pump expression in ornithine transcarbamylase-deficient children.

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Improved liver function and relieved pruritus after 4-phenylbutyrate therapy in a patient with progressive familial intrahepatic cholestasis type 2

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

Liver transcript analysis reveals aberrant splicing due to silent and intronic variations in the ABCB11 gene

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

21 January 2018

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

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

7 January 2021

based on heuristic

inferred from DOI database lookup

A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function

1 reference

https://api.crossref.org/works/10.1002%2FHEP.27767

7 January 2021

based on heuristic

inferred from DOI database lookup

Identifiers

10.1002/HEP.27767

1 reference

Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

14 March 2018

http://europepmc.org/abstract/MED/25716872

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