Rescue of CFTR NBD2 mutants N1303K and S1235R is influenced by the functioning of the autophagosome (Q89217709)

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1 April 2020

title

Rescue of CFTR NBD2 mutants N1303K and S1235R is influenced by the functioning of the autophagosome (English)

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7

Liudmila Cebotaru

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6

William B Guggino

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author name string

Qiangni Liu

1

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Inna Sabirzhanova

2

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Murali K Yanda

3

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Emily A S Bergbower

4

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Clément Boinot

5

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publication date

20 June 2018

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Journal of Cystic Fibrosis

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published in

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volume

17

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issue

5

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page(s)

582-594

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Incidence and expression of the N1303K mutation of the cystic fibrosis (CFTR) gene

1 April 2020

cites work

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12 December 2020

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12 December 2020

Expression and characterization of the cystic fibrosis transmembrane conductance regulator.

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Multiple proteolytic systems, including the proteasome, contribute to CFTR processing.

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The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells

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

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

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Cystic fibrosis as a disease of misprocessing of the cystic fibrosis transmembrane conductance regulator glycoprotein

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Phenotype/genotype correlation and cystic fibrosis related diabetes mellitus (Italian Multicenter Study)

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Molecular dissection of autophagy: two ubiquitin-like systems

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Impairment of the ubiquitin-proteasome system by protein aggregation

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Analysis of the entire coding region of the cystic fibrosis transmembrane regulator gene in idiopathic pancreatitis

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An overview of the pathogenesis of cystic fibrosis lung disease

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Comparison of the CFTR mutation spectrum in three cohorts of patients of Celtic origin from Brittany (France) and Ireland

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CFTR genotypes in patients with normal or borderline sweat chloride levels

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Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.

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Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules

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How to interpret LC3 immunoblotting

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Pharmacological chaperoning: two 'hits' are better than one.

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Autophagy fights disease through cellular self-digestion

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Role of the aggresome pathway in cancer: targeting histone deacetylase 6-dependent protein degradation

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Cl transport in complemented CF bronchial epithelial cells correlates with CFTR mRNA expression levels

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Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

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12 December 2020

HDAC6 controls autophagosome maturation essential for ubiquitin-selective quality-control autophagy

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12 December 2020

Peripheral protein quality control removes unfolded CFTR from the plasma membrane

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12 December 2020

Defective CFTR induces aggresome formation and lung inflammation in cystic fibrosis through ROS-mediated autophagy inhibition

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Cystic fibrosis: A disorder with defective autophagy

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The Beclin 1 network regulates autophagy and apoptosis

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Evaluation of the disease liability of CFTR variants.

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Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.

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p62: a versatile multitasker takes on cancer

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Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on ΔF508 cystic fibrosis transmembrane conductance regulator

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12 December 2020

Mechanism-based corrector combination restores ΔF508-CFTR folding and function

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12 December 2020

Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene

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12 December 2020

Development of a histone deacetylase 6 inhibitor and its biological effects

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12 December 2020

Correcting the cystic fibrosis disease mutant, A455E CFTR

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12 December 2020

Interplay between HDAC6 and its interacting partners: essential roles in the aggresome-autophagy pathway and neurodegenerative diseases

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12 December 2020

Clearance of misfolded and aggregated proteins by aggrephagy and implications for aggregation diseases

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12 December 2020

Rescue of NBD2 mutants N1303K and S1235R of CFTR by small-molecule correctors and transcomplementation

1 reference

12 December 2020

Rescuing Trafficking Mutants of the ATP-binding Cassette Protein, ABCA4, with Small Molecule Correctors as a Treatment for Stargardt Eye Disease.

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12 December 2020

Combination of Correctors Rescue ΔF508-CFTR by Reducing Its Association with Hsp40 and Hsp27.

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12 December 2020

Lumacaftor-ivacaftor (Orkambi) for cystic fibrosis: behind the 'breakthrough'.

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12 December 2020

10.1016/J.JCF.2018.05.016

Identifiers

DOI

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PubMed publication ID

29936070

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