A progressive familial intrahepatic cholestasis type 2 mutation causes an unstable, temperature-sensitive bile salt export pump. (Q40607981)

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22 December 2019

title

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

1 reference

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progressive familial intrahepatic cholestasis

22 December 2019

main subject

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author

Michael Müller

7

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22 December 2019

author name string

Jacqueline R M Plass

1

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22 December 2019

Olaf Mol

2

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22 December 2019

Janette Heegsma

3

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22 December 2019

Mariska Geuken

4

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22 December 2019

Joost de Bruin

5

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22 December 2019

Geeske Elling

6

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22 December 2019

Klaas Nico Faber

8

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22 December 2019

Peter L M Jansen

9

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22 December 2019

publication date

1 January 2004

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22 December 2019

published in

Journal of Hepatology

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22 December 2019

volume

40

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22 December 2019

issue

1

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22 December 2019

page(s)

24-30

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Rat liver canalicular membrane vesicles contain an ATP-dependent bile acid transport system

22 December 2019

cites work

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The sister of P-glycoprotein represents the canalicular bile salt export pump of mammalian liver

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Amino acid substitutions in the first transmembrane domain (TM1) of P-glycoprotein that alter substrate specificity

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Mutations in the MDR3 gene cause progressive familial intrahepatic cholestasis

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

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A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis

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Hepatocanalicular bile salt export pump deficiency in patients with progressive familial intrahepatic cholestasis

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7 January 2021

Farnesoid X receptor and bile salts are involved in transcriptional regulation of the gene encoding the human bile salt export pump

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Stereoselective transport of hydrophilic quaternary drugs by human MDR1 and rat Mdr1b P-glycoproteins

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Up-regulation of the multidrug resistance genes, Mrp1 and Mdr1b, and down-regulation of the organic anion transporter, Mrp2, and the bile salt transporter, Spgp, in endotoxemic rat liver

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Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

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Conformational and temperature-sensitive stability defects of the delta F508 cystic fibrosis transmembrane conductance regulator in post-endoplasmic reticulum compartments.

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7 January 2021

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Identifiers

DOI

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22 December 2019

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