A little CFTR goes a long way: CFTR-dependent sweat secretion from G551D and R117H-5T cystic fibrosis subjects taking ivacaftor (Q31151483)

21 August 2017

A little CFTR goes a long way: CFTR-dependent sweat secretion from G551D and R117H-5T cystic fibrosis subjects taking ivacaftor (English)

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21 August 2017

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Il-Ho Park

4

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author name string

Jessica E Char

1

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21 August 2017

Marlene H Wolfe

2

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21 August 2017

Hyung-Ju Cho

3

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21 August 2017

Jin Hyeok Jeong

5

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21 August 2017

Eric Frisbee

6

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21 August 2017

Colleen Dunn

7

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21 August 2017

Zoe Davies

8

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21 August 2017

Carlos Milla

9

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21 August 2017

Richard B Moss

10

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21 August 2017

Ewart A C Thomas

11

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21 August 2017

Jeffrey J Wine

12

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21 August 2017

language of work or name

English

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publication date

10 February 2014

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21 August 2017

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21 August 2017

9

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21 August 2017

2

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21 August 2017

e88564

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Creative Commons Attribution 4.0 International

21 August 2017

start time

10 February 2014

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April 2022 Public Data File from Crossref

copyright status

copyrighted

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cites work

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Role of tyrosine phosphorylation in the muscarinic activation of the cystic fibrosis transmembrane conductance regulator (CFTR).

21 June 2018

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Vx-770 potentiates CFTR function by promoting decoupling between the gating cycle and ATP hydrolysis cycle

21 June 2018

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Ivacaftor potentiation of multiple CFTR channels with gating mutations

21 June 2018

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A CFTR potentiator in patients with cystic fibrosis and the G551D mutation

21 June 2018

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Mucin variable number tandem repeat polymorphisms and severity of cystic fibrosis lung disease: significant association with MUC5AC.

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Defective fluid secretion from submucosal glands of nasal turbinates from CFTR-/- and CFTR (ΔF508/ΔF508) pigs

21 June 2018

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Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation

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Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs

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Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis.

21 June 2018

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CFTR-adenylyl cyclase I association responsible for UTP activation of CFTR in well-differentiated primary human bronchial cell cultures

21 June 2018

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

21 June 2018

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G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects

21 June 2018

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Polyvariant mutant cystic fibrosis transmembrane conductance regulator genes. The polymorphic (Tg)m locus explains the partial penetrance of the T5 polymorphism as a disease mutation

21 June 2018

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Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype

21 June 2018

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A change in gating mode leading to increased intrinsic Cl- channel activity compensates for defective processing in a cystic fibrosis mutant corresponding to a mild form of the disease

21 June 2018

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Failure to induce secretion in jejunal biopsies from children with cystic fibrosis

21 June 2018

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21 June 2018

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21 June 2018

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21 June 2018

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

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Rapid characterization of the variable length polythymidine tract in the cystic fibrosis (CFTR) gene: association of the 5T allele with selected CFTR mutations and its incidence in atypical sinopulmonary disease

21 January 2018

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Role of cystic fibrosis transmembrane conductance regulator in patients with chronic sinopulmonary disease

21 January 2018

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Submucosal gland dysfunction as a primary defect in cystic fibrosis

21 January 2018

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Statistical issues in studies of individual response

31 October 2018

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12 December 2020

Assessment of CFTR function in homozygous R117H-7T subjects

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12 December 2020

Physiology of the sweat gland in cystic fibrosis of the pancreas

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12 December 2020

Influence of abnormal Cl- impermeability on sweating in cystic fibrosis

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Hyposecretion of beta-adrenergically induced sweating in cystic fibrosis heterozygotes

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Genetic modifiers of lung disease in cystic fibrosis

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12 December 2020

Genetic basis of variable exon 9 skipping in cystic fibrosis transmembrane conductance regulator mRNA

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Correlation of sweat chloride concentration with classes of the cystic fibrosis transmembrane conductance regulator gene mutations

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10.1371/JOURNAL.PONE.0088564

Identifiers

DOI

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21 August 2017

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21 August 2017

PubMed publication ID

24520399

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