Folding defects in P-type ATP 8B1 associated with hereditary cholestasis are ameliorated by 4-phenylbutyrate. (Q43240642)

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12 February 2020

title

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

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12 February 2020

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ChEBI release 2020-05-28

10

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12 February 2020

Joost Holthuis

7

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12 February 2020

author name string

Lieke M van der Velden

1

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12 February 2020

Janneke M Stapelbroek

2

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12 February 2020

Elmar Krieger

3

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12 February 2020

Peter V E van den Berghe

4

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12 February 2020

Ruud Berger

5

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12 February 2020

Patricia M Verhulst

6

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12 February 2020

Roderick H J Houwen

8

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12 February 2020

Leo W J Klomp

9

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12 February 2020

language of work or name

English

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publication date

1 January 2010

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12 February 2020

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12 February 2020

volume

51

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12 February 2020

issue

1

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12 February 2020

page(s)

286-296

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A gene encoding a P-type ATPase mutated in two forms of hereditary cholestasis

12 February 2020

cites work

1 reference

Update on progressive familial intrahepatic cholestasis

21 January 2018

1 reference

A missense mutation in FIC1 is associated with greenland familial cholestasis

21 January 2018

1 reference

Cdc50p, a protein required for polarized growth, associates with the Drs2p P-type ATPase implicated in phospholipid translocation in Saccharomyces cerevisiae

21 January 2018

1 reference

ATP8B1 requires an accessory protein for endoplasmic reticulum exit and plasma membrane lipid flippase activity

21 January 2018

1 reference

Lipid traffic: floppy drives and a superhighway.

21 January 2018

1 reference

FIC1, the protein affected in two forms of hereditary cholestasis, is localized in the cholangiocyte and the canalicular membrane of the hepatocyte

21 January 2018

1 reference

Familial intrahepatic cholestasis 1: studies of localization and function

21 January 2018

1 reference

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

21 January 2018

1 reference

Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency

21 January 2018

1 reference

Characterization of mutations in ATP8B1 associated with hereditary cholestasis

21 January 2018

1 reference

Two established in vitro cell lines from human mesenchymal tumours

21 January 2018

1 reference

Analysis of mutation in human cells by using an Epstein-Barr virus shuttle system

21 January 2018

1 reference

Accurate normalization of real-time quantitative RT-PCR data by geometric averaging of multiple internal control genes

21 January 2018

1 reference

TRPV5 is internalized via clathrin-dependent endocytosis to enter a Ca2+-controlled recycling pathway

21 January 2018

1 reference

The recognition and retrotranslocation of misfolded proteins from the endoplasmic reticulum

21 January 2018

1 reference

Reduced hepatic expression of farnesoid X receptor in hereditary cholestasis associated to mutation in ATP8B1

21 January 2018

1 reference

ATP8B1 mutations in British cases with intrahepatic cholestasis of pregnancy.

21 January 2018

1 reference

Nasobiliary drainage induces long-lasting remission in benign recurrent intrahepatic cholestasis

21 January 2018

1 reference

FIC1 and BSEP defects in Taiwanese patients with chronic intrahepatic cholestasis with low γ-glutamyltranspeptidase levels

21 January 2018

1 reference

Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis

21 January 2018

1 reference

inferred from DOI database lookup

7 January 2021

based on heuristic

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

1 reference

inferred from DOI database lookup

7 January 2021

based on heuristic

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