Cystic Fibrosis Transmembrane Conductance Regulator
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Subject Areas on Research
- 5' exon replacement and repair by spliceosome-mediated RNA trans-splicing.
- A C-terminal motif found in the beta2-adrenergic receptor, P2Y1 receptor and cystic fibrosis transmembrane conductance regulator determines binding to the Na+/H+ exchanger regulatory factor family of PDZ proteins.
- A complex intronic enhancer regulates expression of the CFTR gene by direct interaction with the promoter.
- A cystic fibrosis transmembrane conductance regulator splice variant with partial penetrance associated with variable cystic fibrosis presentations.
- A novel mutation in the cystic fibrosis gene in patients with pulmonary disease but normal sweat chloride concentrations.
- A revised airway epithelial hierarchy includes CFTR-expressing ionocytes.
- A yeast metal resistance protein similar to human cystic fibrosis transmembrane conductance regulator (CFTR) and multidrug resistance-associated protein.
- ATP hydrolysis by a CFTR domain: pharmacology and effects of G551D mutation.
- Activation of G551D CFTR channel with MPB-91: regulation by ATPase activity and phosphorylation.
- Adenovirus-mediated transfer of the CFTR gene to lung of nonhuman primates: biological efficacy study.
- Adenovirus-mediated transfer of the CFTR gene to lung of nonhuman primates: toxicity study.
- Agonist-induced coordinated trafficking of functionally related transport proteins for water and ions in cholangiocytes.
- An approach for treating the hepatobiliary disease of cystic fibrosis by somatic gene transfer.
- Aquagenic wrinkling: a unique facial presentation.
- Are mutations in the cystic fibrosis gene important in chronic pancreatitis?
- Assembly of signaling complexes by the sodium-hydrogen exchanger regulatory factor family of PDZ-containing proteins.
- Assessment of the CFTR and ENaC association.
- Bcl-2 suppresses sarcoplasmic/endoplasmic reticulum Ca2+-ATPase expression in cystic fibrosis airways: role in oxidant-mediated cell death.
- Biochemical and biophysical identification of cystic fibrosis transmembrane conductance regulator chloride channels as components of endocytic clathrin-coated vesicles.
- CFTR as a cAMP-dependent regulator of sodium channels.
- CFTR haplotype analysis reveals genetic heterogeneity in the etiology of congenital bilateral aplasia of the vas deferens.
- CFTR regulation of epithelial sodium channel.
- CFTR: development of high- affinity antibodies and localization in sweat gland.
- Carrier screening for cystic fibrosis in US genetic testing laboratories: a survey of laboratory directors.
- Cellular and viral splicing factors can modify the splicing pattern of CFTR transcripts carrying splicing mutations.
- Cftr controls lumen expansion and function of Kupffer's vesicle in zebrafish.
- Characterization of the cystic fibrosis transmembrane conductance regulator in a colonocyte cell line.
- Characterization of the cystic fibrosis transmembrane conductance regulator promoter region. Chromatin context and tissue-specificity.
- Cl- and K+ transport in human biliary cell lines.
- Computational design of a PDZ domain peptide inhibitor that rescues CFTR activity.
- Control of cellular GADD34 levels by the 26S proteasome.
- Correction of aberrant splicing of the cystic fibrosis transmembrane conductance regulator (CFTR) gene by antisense oligonucleotides.
- Cse1l is a negative regulator of CFTR-dependent fluid secretion.
- Cystic fibrosis in Jews: frequency and mutation distribution.
- Cystic fibrosis in the pancreas: recent advances provide new insights.
- Cystic fibrosis mutations and genetic predisposition to idiopathic chronic pancreatitis.
- Cystic fibrosis transmembrane conductance regulator activation is reduced in the small intestine of Na+/H+ exchanger 3 regulatory factor 1 (NHERF-1)- but Not NHERF-2-deficient mice.
- Cystic fibrosis transmembrane regulator regulates uptake of sphingoid base phosphates and lysophosphatidic acid: modulation of cellular activity of sphingosine 1-phosphate.
- Development of rAAV2-CFTR: History of the First rAAV Vector Product to be Used in Humans.
- Differential contribution of cis-regulatory elements to higher order chromatin structure and expression of the CFTR locus.
- Direct gene transfer of human CFTR into human bronchial epithelia of xenografts with E1-deleted adenoviruses.
- Dysregulation of proteoglycan production by intrahepatic biliary epithelial cells bearing defective (delta-f508) cystic fibrosis transmembrane conductance regulator.
- ENaC- and CFTR-dependent ion and fluid transport in mammary epithelia.
- Efficient intratracheal delivery of airway epithelial cells in mice and pigs.
- Expression of an abundant alternatively spliced form of the cystic fibrosis transmembrane conductance regulator (CFTR) gene is not associated with a cAMP-activated chloride conductance.
- Expression of the cystic fibrosis gene in adult human lung.
- Functional analysis of a mammalian odorant receptor subfamily.
- Gene targeting of a CFTR allele in HT29 human epithelial cells.
- Gene therapy for cystic fibrosis using E1-deleted adenovirus: a phase I trial in the nasal cavity. The University of North Carolina at Chapel Hill.
- Gene therapy of cystic fibrosis lung disease using E1 deleted adenoviruses: a phase I trial.
- Generation of renewable mouse intestinal epithelial cell monolayers and organoids for functional analyses.
- Genetic prevalence and characteristics in children with recurrent pancreatitis.
- Gq activity- and β-arrestin-1 scaffolding-mediated ADGRG2/CFTR coupling are required for male fertility.
- Heritability of respiratory infection with Pseudomonas aeruginosa in cystic fibrosis.
- Hsp 70/Hsp 90 organizing protein as a nitrosylation target in cystic fibrosis therapy.
- Human cystic fibrosis transmembrane conductance regulator directed to respiratory epithelial cells of transgenic mice.
- Hypoxia inducible factor-1 (HIF-1)-mediated repression of cystic fibrosis transmembrane conductance regulator (CFTR) in the intestinal epithelium.
- Idiopathic pancreatitis related to CFTR: complex inheritance and identification of a modifier gene.
- Inactivation of E2a in recombinant adenoviruses improves the prospect for gene therapy in cystic fibrosis.
- Intron-8 polythymidine sequence in Australasian individuals with CF mutations R117H and R117C.
- Intronic enhancers coordinate epithelial-specific looping of the active CFTR locus.
- Localization of the cystic fibrosis transmembrane conductance regulator in human bile duct epithelial cells.
- Localization of the cystic fibrosis transmembrane conductance regulator in pancreas.
- Loss of cftr function leads to pancreatic destruction in larval zebrafish.
- Low temperature and chemical rescue affect molecular proximity of DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) and epithelial sodium channel (ENaC).
- Messenger RNA repair and restoration of protein function by spliceosome-mediated RNA trans-splicing.
- Molecular basis of defective anion transport in L cells expressing recombinant forms of CFTR.
- Molecular proximity of cystic fibrosis transmembrane conductance regulator and epithelial sodium channel assessed by fluorescence resonance energy transfer.
- Motion--genetic testing is useful in the diagnosis of nonhereditary pancreatic conditions: arguments against the motion.
- Multicenter characterization and validation of the intron-8 poly(T) tract (IVS8-T) status in 25 Coriell cell repository cystic fibrosis reference cell lines for cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation assays.
- Mutations of the cystic fibrosis gene and pancreatitis.
- Novel human bronchial epithelial cell lines for cystic fibrosis research.
- Partial correction of endogenous DeltaF508 CFTR in human cystic fibrosis airway epithelia by spliceosome-mediated RNA trans-splicing.
- Pathophysiology of SPINK mutations in pancreatic development and disease.
- Phosphorylation of the cystic fibrosis transmembrane conductance regulator.
- Polarized expression of cAMP-activated chloride channels in isolated epithelial cells.
- Protein kinase A regulates ATP hydrolysis and dimerization by a CFTR (cystic fibrosis transmembrane conductance regulator) domain.
- Protein kinase C and Ca2+ activation of mucin secretion in airway goblet cells.
- Protein synthesis inhibitors and the chemical chaperone TMAO reverse endoplasmic reticulum perturbation induced by overexpression of the iodide transporter pendrin.
- Reduced CFTR function and the pathobiology of idiopathic pancreatitis.
- Regulation of membrane chloride currents in rat bile duct epithelial cells.
- Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis.
- Repair of CFTR mRNA by spliceosome-mediated RNA trans-splicing.
- Restoration of the cystic fibrosis transmembrane conductance regulator function by splicing modulation.
- S-nitrosylating agents: a novel class of compounds that increase cystic fibrosis transmembrane conductance regulator expression and maturation in epithelial cells.
- Salt stress increases abundance and glycosylation of CFTR localized at apical surfaces of salt gland secretory cells.
- Screening of CFTR mutations in an isolated population: identification of carriers and patients.
- Sequences within the coding regions of clotting factor VIII and CFTR block transcriptional elongation.
- Structural cues involved in endoplasmic reticulum degradation of G85E and G91R mutant cystic fibrosis transmembrane conductance regulator.
- Submucosal glands are the predominant site of CFTR expression in the human bronchus.
- Survey of cystic fibrosis transmembrane conductance regulator genotypes in primary sclerosing cholangitis.
- The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment.
- The deacetylase HDAC6 regulates aggresome formation and cell viability in response to misfolded protein stress.
- The impact of cystic fibrosis and PSTI/SPINK1 gene mutations on susceptibility to chronic pancreatitis.
- The molecular basis of disease variability among cystic fibrosis patients carrying the 3849+10 kb C-->T mutation.
- The role of cystic fibrosis gene mutations in determining susceptibility to chronic pancreatitis.
- Tonically active protein kinase A regulates neurotransmitter release at the squid giant synapse.
- Ultrastructural localization of variant forms of cystic fibrosis transmembrane conductance regulator in human bronchial epithelial of xenografts.
- Variable levels of normal RNA in different fetal organs carrying a cystic fibrosis transmembrane conductance regulator splicing mutation.
- 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.
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Keywords of People
- Jowell, Paul Simon, Professor of Medicine, Medicine, Gastroenterology