Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype.
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The human DnaJ homologue (Hdj)-1/heat-shock protein (Hsp) 40 co-chaperone is required for the in vivo stabilization of the cystic fibrosis transmembrane conductance regulator by Hsp70Rectal forceps biopsy procedure in cystic fibrosis: technical aspects and patients perspective for clinical trials feasibility.A little CFTR goes a long way: CFTR-dependent sweat secretion from G551D and R117H-5T cystic fibrosis subjects taking ivacaftorVery mild disease phenotype of congenic CftrTgH(neoim)Hgu cystic fibrosis miceIn vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies.A mild variant of cystic fibrosis.Bestrophin-2 mediates bicarbonate transport by goblet cells in mouse colonDeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis.Intestinal current measurement versus nasal potential difference measurements for diagnosis of cystic fibrosis: a case-control study.Establishing a diagnosis of cystic fibrosis.Electrolyte transport in the mammalian colon: mechanisms and implications for disease.In vivo readout of CFTR function: ratiometric measurement of CFTR-dependent secretion by individual, identifiable human sweat glandsSome gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.CFTR and differentiation markers expression in non-CF and delta F 508 homozygous CF nasal epithelium.Chloride conductance and genetic background modulate the cystic fibrosis phenotype of Delta F508 homozygous twins and siblings.Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutationA delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo.Deep resequencing of CFTR in 762 F508del homozygotes reveals clusters of non-coding variants associated with cystic fibrosis disease traits.Bioelectric characterization of epithelia from neonatal CFTR knockout ferrets.Mechanism of dysfunction of two nucleotide binding domain mutations in cystic fibrosis transmembrane conductance regulator that are associated with pancreatic sufficiency.Mis-trafficking of bicarbonate transporters: implications to human diseases.Gastroenterological endpoints in drug trials for cystic fibrosis.Individualized medicine using intestinal responses to CFTR potentiators and correctors.A mouse model for the cystic fibrosis delta F508 mutation.Cystic fibrosis F508del patients have apically localized CFTR in a reduced number of airway cells.Inhibition of intestinal Cl- secretion by clotrimazole: direct effect on basolateral membrane K+ channels.Psoralens: novel modulators of Cl- secretion.Acetylcholine induces cytosolic Ca2+ mobilization in isolated distal colonic crypts from normal and cystic fibrosis mice.Evaluation of the intestinal current measurement method as a diagnostic test for cystic fibrosis.Structural abnormalities in islets from very young children with cystic fibrosis may contribute to cystic fibrosis-related diabetes.Nasal airway ion transport and lung function in young people with cystic fibrosis.Expression of delta F508 cystic fibrosis transmembrane conductance regulator protein and related chloride transport properties in the gallbladder epithelium from cystic fibrosis patients.Cystic fibrosis transmembrane conductance regulator (CFTR)-mediated residual chloride secretion does not protect against early chronic Pseudomonas aeruginosa infection in F508del homozygous cystic fibrosis patients.
P2860
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P2860
Determinants of mild clinical symptoms in cystic fibrosis patients. Residual chloride secretion measured in rectal biopsies in relation to the genotype.
description
1994 nî lūn-bûn
@nan
1994 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
1994 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
1994年の論文
@ja
1994年論文
@yue
1994年論文
@zh-hant
1994年論文
@zh-hk
1994年論文
@zh-mo
1994年論文
@zh-tw
1994年论文
@wuu
name
Determinants of mild clinical ...... s in relation to the genotype.
@ast
Determinants of mild clinical ...... s in relation to the genotype.
@en
Determinants of mild clinical ...... s in relation to the genotype.
@nl
type
label
Determinants of mild clinical ...... s in relation to the genotype.
@ast
Determinants of mild clinical ...... s in relation to the genotype.
@en
Determinants of mild clinical ...... s in relation to the genotype.
@nl
prefLabel
Determinants of mild clinical ...... s in relation to the genotype.
@ast
Determinants of mild clinical ...... s in relation to the genotype.
@en
Determinants of mild clinical ...... s in relation to the genotype.
@nl
P2093
P2860
P356
P1476
Determinants of mild clinical ...... s in relation to the genotype.
@en
P2093
Sinaasappel M
de Jonge HR
de Jongste JC
P2860
P304
P356
10.1172/JCI116993
P407
P577
1994-02-01T00:00:00Z