Expression of the cystic fibrosis gene in non-epithelial invertebrate cells produces a regulated anion conductance.
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Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasomeIdentification of a protein that confers calcitonin gene-related peptide responsiveness to oocytes by using a cystic fibrosis transmembrane conductance regulator assayPathophysiology of gene-targeted mouse models for cystic fibrosisMouse cystic fibrosis transmembrane conductance regulator forms cAMP-PKA-regulated apical chloride channels in cortical collecting ductLung infections associated with cystic fibrosis.Perturbation of the pore of the cystic fibrosis transmembrane conductance regulator (CFTR) inhibits its atpase activity.Abnormal secretagogue-induced intracellular free Ca2+ regulation in cystic fibrosis nasal epithelial cellsNine cystic fibrosis patients homozygous for the CFTR nonsense mutation R1162X have mild or moderate lung disease.CFTR transcripts are undetectable in lymphocytes and respiratory epithelial cells of a CF patient homozygous for the nonsense mutation R553X.Cystic fibrosis: exploiting its genetic basis in the hunt for new therapiesPhenotypic profiling of CFTR modulators in patient-derived respiratory epithelia.Cystic fibrosis transmembrane conductance regulator interacts with multiple immunoglobulin domains of filamin A.Nonsense mutation R1162X of the cystic fibrosis transmembrane conductance regulator gene does not reduce messenger RNA expression in nasal epithelial tissue.Phosphorylation of CFTR by PKA promotes binding of the regulatory domainRegulation of CFTR expression and function during differentiation of intestinal epithelial cells.Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent manner.Structural and functional similarities between the nucleotide-binding domains of CFTR and GTP-binding proteinsThe cystic fibrosis transmembrane conductance regulator chloride channel. Iodide block and permeation.A novel exon in the cystic fibrosis transmembrane conductance regulator gene activated by the nonsense mutation E92X in airway epithelial cells of patients with cystic fibrosisInteraction between permeation and gating in a putative pore domain mutant in the cystic fibrosis transmembrane conductance regulator.The cystic fibrosis transmembrane conductance regulator impedes proteolytic stimulation of the epithelial Na+ channel.Identification and regulation of the cystic fibrosis transmembrane conductance regulator-generated chloride channelSevere deficiency of cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis.Molecular analysis of the ovine cystic fibrosis transmembrane conductance regulator gene.Interleukin-13 (IL-13)/IL-13 receptor alpha1 (IL-13Ralpha1) signaling regulates intestinal epithelial cystic fibrosis transmembrane conductance regulator channel-dependent Cl- secretion.Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium.Transfer of a constitutive viral promoter-cystic fibrosis transmembrane conductance regulator cDNA to human epithelial cells conveys resistance to down-regulation of cAMP-regulated Cl- secretion in the presence of inflammatory stimuli.Identification of an ion channel-forming motif in the primary structure of CFTR, the cystic fibrosis chloride channelExpression of the human cystic fibrosis transmembrane conductance regulator gene in the mouse lung after in vivo intratracheal plasmid-mediated gene transfer.Genetic determination of exocrine pancreatic function in cystic fibrosisIdentification of a nonframeshift 84-bp deletion in exon 13 of the cystic fibrosis gene.Evolution of an ion-translocating ATPase.Altered protein folding may be the molecular basis of most cases of cystic fibrosis.Energy metabolism in cystic fibrosis.Normalization of raised sodium absorption and raised calcium-mediated chloride secretion by adenovirus-mediated expression of cystic fibrosis transmembrane conductance regulator in primary human cystic fibrosis airway epithelial cells.Molecular consequences of cystic fibrosis transmembrane regulator (CFTR) gene mutations in the exocrine pancreas.Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epitheliacAMP stimulates bicarbonate secretion across normal, but not cystic fibrosis airway epithelia.Neutrophil elastase in respiratory epithelial lining fluid of individuals with cystic fibrosis induces interleukin-8 gene expression in a human bronchial epithelial cell line.Phosphatase inhibitors activate normal and defective CFTR chloride channels
P2860
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P2860
Expression of the cystic fibrosis gene in non-epithelial invertebrate cells produces a regulated anion conductance.
description
1991 nî lūn-bûn
@nan
1991年の論文
@ja
1991年論文
@yue
1991年論文
@zh-hant
1991年論文
@zh-hk
1991年論文
@zh-mo
1991年論文
@zh-tw
1991年论文
@wuu
1991年论文
@zh
1991年论文
@zh-cn
name
Expression of the cystic fibro ...... a regulated anion conductance.
@en
type
label
Expression of the cystic fibro ...... a regulated anion conductance.
@en
prefLabel
Expression of the cystic fibro ...... a regulated anion conductance.
@en
P2093
P1433
P1476
Expression of the cystic fibro ...... a regulated anion conductance.
@en
P2093
Ackerley CA
Hanrahan JW
Naismith AL
Rommens JM
P304
P356
10.1016/0092-8674(91)90498-N
P407
P577
1991-02-01T00:00:00Z