(Q24308743)

2

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Martina Wilke

3

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Marcel Bijvelds

4

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Huub Jorna

5

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Patricia Melin

6

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Hugo DeJonge

7

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Frederic Becq

The cystic fibrosis transmembrane conductance regulator: an intriguing protein with pleiotropic functions.

8

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language of work or name

English

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publication date

3 April 2006

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published in

FEBS Letters

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volume

580

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page(s)

2081-6

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issue

8

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cites work

1 reference

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Phosphorylation-regulated Cl- channel in CHO cells stably expressing the cystic fibrosis gene.

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Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis.

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Quality control in the endoplasmic reticulum

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Roles of N-linked glycans in the endoplasmic reticulum

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The molecular chaperone calnexin binds Glc1Man9GlcNAc2 oligosaccharide as an initial step in recognizing unfolded glycoproteins

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Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects.

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Delta F508 CFTR pool in the endoplasmic reticulum is increased by calnexin overexpression.

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Calnexin Delta 185-520 partially reverses the misprocessing of the Delta F508 cystic fibrosis transmembrane conductance regulator.

21 January 2018

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The role of the iminosugar N-butyldeoxynojirimycin (miglustat) in the management of type I (non-neuronopathic) Gaucher disease: a position statement

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Targeting glycosylation as a therapeutic approach.

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A1 adenosine-receptor antagonists activate chloride efflux from cystic fibrosis cells.

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The cystic fibrosis mutation G1349D within the signature motif LSHGH of NBD2 abolishes the activation of CFTR chloride channels by genistein.

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A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo.

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Animal models of cystic fibrosis.

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Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers.

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The severe G480C cystic fibrosis mutation, when replicated in the mouse, demonstrates mistrafficking, normal survival and organ-specific bioelectrics.

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

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Role of N-linked oligosaccharide recognition, glucose trimming, and calnexin in glycoprotein folding and quality control

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Traffic jams II: an update of diseases of intracellular transport.

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Novel mechanism for Brugada syndrome: defective surface localization of an SCN5A mutant (R1432G).

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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.

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