A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo.
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Five Percent of Normal Cystic Fibrosis Transmembrane Conductance Regulator mRNA Ameliorates the Severity of Pulmonary Disease in Cystic FibrosisRescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustatA functional CFTR assay using primary cystic fibrosis intestinal organoidsPathophysiology of gene-targeted mouse models for cystic fibrosisMyotonia congenita mutation enhances the degradation of human CLC-1 chloride channelsPseudomonas aeruginosa inhibits endocytic recycling of CFTR in polarized human airway epithelial cellsSphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) ActivityStudies of mucus in mouse stomach, small intestine, and colon. I. Gastrointestinal mucus layers have different properties depending on location as well as over the Peyer's patchesSpontaneous rescue from cystic fibrosis in a mouse model.Loss of inositol 1,4,5-trisphosphate receptors from bile duct epithelia is a common event in cholestasis.The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in maturation stage ameloblasts, odontoblasts and bone cellsElectrophysiological evidence for the presence of cystic fibrosis transmembrane conductance regulator (CFTR) in mouse sperm.DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis.Defective CFTR leads to aberrant β-catenin activation and kidney fibrosis.AAV exploits subcellular stress associated with inflammation, endoplasmic reticulum expansion, and misfolded proteins in models of cystic fibrosis.Pathophysiological preconditions promoting mixed "black" pigment plus cholesterol gallstones in a DeltaF508 mouse model of cystic fibrosis.Cysteine string protein interacts with and modulates the maturation of the cystic fibrosis transmembrane conductance regulator.Ion channel associated diseases: overview of molecular mechanismsThe gastrointestinal mucus system in health and diseaseCysteine string protein monitors late steps in cystic fibrosis transmembrane conductance regulator biogenesis.Rescue of murine F508del CFTR activity in native intestine by low temperature and proteasome inhibitorsMulticenter intestinal current measurements in rectal biopsies from CF and non-CF subjects to monitor CFTR function.Hypertonic saline releases the attached small intestinal cystic fibrosis mucusThe role of epithelial P2Y2 and P2Y4 receptors in the regulation of intestinal chloride secretion.ERp29 regulates DeltaF508 and wild-type cystic fibrosis transmembrane conductance regulator (CFTR) trafficking to the plasma membrane in cystic fibrosis (CF) and non-CF epithelial cells.Loss of Slc26a9 anion transporter alters intestinal electrolyte and HCO3(-) transport and reduces survival in CFTR-deficient miceN-terminal transmembrane domain of the SUR controls trafficking and gating of Kir6 channel subunits.Role of IL-1β in experimental cystic fibrosis upon P. aeruginosa infection.Cystic fibrosis mouse model-dependent intestinal structure and gut microbiomeGenetic Inhibition Of The Ubiquitin Ligase Rnf5 Attenuates Phenotypes Associated To F508del Cystic Fibrosis Mutation.Processing and function of CFTR-DeltaF508 are species-dependent.5'-adenosine monophosphate mediated cooling treatment enhances ΔF508-Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) stability in vivo.In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epitheliumOuabain Mimics Low Temperature Rescue of F508del-CFTR in Cystic Fibrosis Epithelial Cells.COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit codeComparative biology of cystic fibrosis animal modelsWhat have we learned from mouse models for cystic fibrosis?Animal models of chronic lung infection with Pseudomonas aeruginosa: useful tools for cystic fibrosis studies.Vasoactive intestinal peptide, forskolin, and genistein increase apical CFTR trafficking in the rectal gland of the spiny dogfish, Squalus acanthias. Acute regulation of CFTR trafficking in an intact epithelium.Cl- absorption across the thick ascending limb is not altered in cystic fibrosis mice. A role for a pseudo-CFTR Cl- channel
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P2860
A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo.
description
article científic
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article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on September 1996
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vedecký článok
@sk
vetenskaplig artikel
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videnskabelig artikel
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vědecký článek
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name
A delta F508 mutation in mouse ...... ive processing defect in vivo.
@en
A delta F508 mutation in mouse ...... ive processing defect in vivo.
@nl
type
label
A delta F508 mutation in mouse ...... ive processing defect in vivo.
@en
A delta F508 mutation in mouse ...... ive processing defect in vivo.
@nl
prefLabel
A delta F508 mutation in mouse ...... ive processing defect in vivo.
@en
A delta F508 mutation in mouse ...... ive processing defect in vivo.
@nl
P2093
P2860
P356
P1476
A delta F508 mutation in mouse ...... ive processing defect in vivo.
@en
P2093
Scholte BJ
de Jonge HR
van Doorninck JH
P2860
P304
P356
10.1172/JCI118917
P407
P577
1996-09-01T00:00:00Z