| scholarly article | Q13442814 |
| P356 | DOI | 10.1126/SCIENCE.7527588 |
| P698 | PubMed publication ID | 7527588 |
| P2093 | author name string | L Zhou | |
| R A Frizzell | |||
| S E Wert | |||
| J A Whitsett | |||
| C R Dey | |||
| M D DuVall | |||
| P433 | issue | 5191 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | cystic fibrosis | Q178194 |
| P304 | page(s) | 1705-1708 | |
| P577 | publication date | 1994-12-01 | |
| P1433 | published in | Science | Q192864 |
| P1476 | title | Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR | |
| P478 | volume | 266 |
| Q24644767 | A guide to Ussing chamber studies of mouse intestine |
| Q37591319 | A mast cell-ILC2-Th9 pathway promotes lung inflammation in cystic fibrosis |
| Q37698433 | A role for CFTR in the elevation of glutathione levels in the lung by oral glutathione administration |
| Q40895642 | A synthetic channel-forming peptide induces Cl(-) secretion: modulation by Ca(2+)-dependent K(+) channels |
| Q40075329 | Acute intratracheal Pseudomonas aeruginosa infection in cystic fibrosis mice is age-independent. |
| Q39580195 | Adenovirus-mediated persistent cystic fibrosis transmembrane conductance regulator expression in mouse airway epithelium |
| Q54243366 | Airway disease phenotypes in animal models of cystic fibrosis. |
| Q60920753 | Animal Models in the Pathophysiology of Cystic Fibrosis |
| Q57113804 | Animal models for cystic fibrosis liver disease (CFLD) |
| Q37289276 | Animal models for target diseases in gene therapy--using DNA and siRNA delivery strategies. |
| Q35178642 | Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology. |
| Q37352252 | Animal models of human disease for gene therapy |
| Q35279508 | CFTR and sphingolipids mediate hypoxic pulmonary vasoconstriction |
| Q39957077 | CFTR expression from a BAC carrying the complete human gene and associated regulatory elements. |
| Q34067800 | CFTR is a pattern recognition molecule that extracts Pseudomonas aeruginosa LPS from the outer membrane into epithelial cells and activates NF-kappa B translocation |
| Q42018121 | CFTR is required for PKA-regulated ATP sensitivity of Kir1.1 potassium channels in mouse kidney |
| Q34399667 | CFTR regulates early pathogenesis of chronic obstructive lung disease in βENaC-overexpressing mice |
| Q92219890 | Calcium-activated chloride channel regulator 1 (CLCA1): More than a regulator of chloride transport and mucus production |
| Q43074262 | Cardiomyocytes with disrupted CFTR function require CaMKII and Ca(2+)-activated Cl(-) channel activity to maintain contraction rate |
| Q35097413 | Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model |
| Q36744020 | Comparative biology of cystic fibrosis animal models |
| Q40858815 | Comparison of the gating behaviour of human and murine cystic fibrosis transmembrane conductance regulator Cl- channels expressed in mammalian cells |
| Q42183922 | Complementation of null CF mice with a human CFTR YAC transgene |
| Q35850895 | Computed Tomography (CT) Scanning Facilitates Early Identification of Neonatal Cystic Fibrosis Piglets |
| Q51725452 | Conditional gene expression in the respiratory epithelium of the mouse. |
| Q39017146 | Cystic Fibrosis and the Nervous System. |
| Q35216745 | Cystic fibrosis growth retardation is not correlated with loss of Cftr in the intestinal epithelium |
| Q35844736 | Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype-phenotype correlations. |
| Q33738695 | Cystic fibrosis transmembrane conductance regulator regulates epithelial cell response to Aspergillus and resultant pulmonary inflammation |
| Q34582621 | Cystic fibrosis transmembrane conductance regulator with a shortened R domain rescues the intestinal phenotype of CFTR-/- mice. |
| Q37343771 | Defective acid sphingomyelinase pathway with Pseudomonas aeruginosa infection in cystic fibrosis |
| Q44599678 | Delivery of CFTR by adenoviral vector to cystic fibrosis mouse lung in a model of chronicPseudomonas aeruginosalung infection |
| Q34987529 | Disease genes: flattery and deception |
| Q34090196 | Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis. |
| Q55399442 | Ductal Mucus Obstruction and Reduced Fluid Secretion Are Early Defects in Chronic Pancreatitis. |
| Q33863162 | Effect of allergy and inflammation on eicosanoid gene expression in CFTR deficiency |
| Q45088980 | Effects of creatine supplementation in cystic fibrosis: results of a pilot study. |
| Q36051231 | Efficacy of the Novel Antibiotic POL7001 in Preclinical Models of Pseudomonas aeruginosa Pneumonia. |
| Q42102291 | Endogenous expression of type II cGMP-dependent protein kinase mRNA and protein in rat intestine. Implications for cystic fibrosis transmembrane conductance regulator |
| Q54621653 | Enhanced IgE allergic response to Aspergillus fumigatus in CFTR-/- mice. |
| Q56970609 | Expression and function of Anoctamin 1/TMEM16A calcium-activated chloride channels in airways of in vivo mouse models for cystic fibrosis research |
| Q45869605 | Expression of CFTR from a ciliated cell-specific promoter is ineffective at correcting nasal potential difference in CF mice |
| Q48704201 | Expression of a brain-type cannabinoid receptor (CB1) in alveolar Type II cells in the lung: regulation by hydrocortisone |
| Q35887545 | Expression of the poliovirus receptor in intestinal epithelial cells is not sufficient to permit poliovirus replication in the mouse gut. |
| Q42217358 | Gene therapy in gastroenterology |
| Q47281671 | Generation of Induced Progenitor-like Cells from Mature Epithelial Cells Using Interrupted Reprogramming |
| Q40908349 | Generation of mice expressing the human glucagon receptor with a direct replacement vector |
| Q64102762 | Genetic association and transcriptome integration identify contributing genes and tissues at cystic fibrosis modifier loci |
| Q34982862 | Genetic therapies for cystic fibrosis lung disease |
| Q41015188 | Heterologous expression systems for study of cystic fibrosis transmembrane conductance regulator. |
| Q37576093 | Hyperglycemia impedes lung bacterial clearance in a murine model of cystic fibrosis-related diabetes |
| Q34531184 | Hypersusceptibility of cystic fibrosis mice to chronic Pseudomonas aeruginosa oropharyngeal colonization and lung infection |
| Q36690556 | IL-1 receptor antagonist ameliorates inflammasome-dependent inflammation in murine and human cystic fibrosis |
| Q28544789 | IL-37 inhibits inflammasome activation and disease severity in murine aspergillosis |
| Q37727368 | Impaired defenses of neonatal mouse alveolar macrophage with cftr deletion are modulated by glutathione and TGFβ1 |
| Q40431922 | In vivo alterations of IFN regulatory factor-1 and PIAS1 protein levels in cystic fibrosis epithelium |
| Q36382475 | In vivo analysis of cadherin function in the mouse intestinal epithelium: essential roles in adhesion, maintenance of differentiation, and regulation of programmed cell death |
| Q48462525 | In-frame elimination of exon 10 in Cftrtm1Unc CF mice |
| Q60015366 | Inhaled bacteriophage-loaded polymeric microparticles ameliorate acute lung infections |
| Q35973303 | Inhibition of high-mobility group box 1 protein (HMGB1) enhances bacterial clearance and protects against Pseudomonas Aeruginosa pneumonia in cystic fibrosis. |
| Q24548775 | Innate lung defenses and compromised Pseudomonas aeruginosa clearance in the malnourished mouse model of respiratory infections in cystic fibrosis |
| Q36891023 | Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs |
| Q40409394 | Knockouts. Targeting the mouse genome: a compendium of knockouts (Part I) |
| Q41819454 | Lack of cystic fibrosis transmembrane conductance regulator in CD3+ lymphocytes leads to aberrant cytokine secretion and hyperinflammatory adaptive immune responses |
| Q34223037 | Left ventricular and aortic dysfunction in cystic fibrosis mice |
| Q37144558 | Limited restoration of cystic fibrosis lung epithelium in vivo with adult bone marrow-derived cells |
| Q89129500 | Lipid Nanoparticle-Delivered Chemically Modified mRNA Restores Chloride Secretion in Cystic Fibrosis |
| Q34414598 | Longevity and plasticity of CFTR provide an argument for noncanonical SNP organization in hominid DNA |
| Q36977287 | Lubiprostone activates non-CFTR-dependent respiratory epithelial chloride secretion in cystic fibrosis mice. |
| Q35840336 | MRP4 and CFTR in the regulation of cAMP and β-adrenergic contraction in cardiac myocytes |
| Q29615286 | Microbial pathogenesis in cystic fibrosis: mucoid Pseudomonas aeruginosa and Burkholderia cepacia |
| Q37098621 | Mitochondrial oxidative stress in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice |
| Q41671586 | Modelling cystic fibrosis in the mouse |
| Q41226239 | Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor |
| Q33731233 | Modulation of exaggerated-IgE allergic responses by gene transfer-mediated antagonism of IL-13 and IL-17e |
| Q37887172 | Mouse models of cystic fibrosis: phenotypic analysis and research applications |
| Q39669861 | N-glycosylation augmentation of the cystic fibrosis epithelium improves Pseudomonas aeruginosa clearance |
| Q37381811 | Na-K-2Cl cotransporter gene expression and function during enterocyte differentiation. Modulation of Cl- secretory capacity by butyrate |
| Q36679834 | New animal models of cystic fibrosis: what are they teaching us? |
| Q41720801 | Novel role of cystic fibrosis transmembrane conductance regulator in maintaining adult mouse olfactory neuronal homeostasis |
| Q41630708 | PATHOPHYSIOLOGIC EVALUATION OF THE TRANSGENIC CFTR "GUT-CORRECTED" PORCINE MODEL OF CYSTIC FIBROSIS. |
| Q36424575 | Pancreatic pathophysiology in cystic fibrosis |
| Q36277502 | Partial Restoration of CFTR Function in cftr-Null Mice following Targeted Cell Replacement Therapy |
| Q77111297 | Pathology of pancreatic and intestinal disorders in cystic fibrosis |
| Q35161835 | Phenotyping mouse pulmonary function in vivo with the lung diffusing capacity. |
| Q28080906 | Precision Genomic Medicine in Cystic Fibrosis |
| Q35991100 | Processing and function of CFTR-DeltaF508 are species-dependent |
| Q37162444 | Reduced NHE3-mediated Na+ absorption increases survival and decreases the incidence of intestinal obstructions in cystic fibrosis mice |
| Q93060190 | Reduced expression of the Ion channel CFTR contributes to airspace enlargement as a consequence of aging and in response to cigarette smoke in mice |
| Q33988505 | Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections |
| Q45402489 | Sendai virus-mediated CFTR gene transfer to the airway epithelium. |
| Q71698850 | Symptoms in a 'leaky' mouse |
| Q33799590 | Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor |
| Q43671300 | Targeting expression of a transgene to the airway surface epithelium using a ciliated cell-specific promoter |
| Q35808789 | The Effects of CFTR and Mucoid Phenotype on Susceptibility and Innate Immune Responses in a Mouse Model of Pneumococcal Lung Disease. |
| Q73280831 | The T3(b) gene promoter directs intestinal epithelial cell-specific expression in transgenic mice |
| Q38023386 | The challenges and promises of new therapies for cystic fibrosis |
| Q41625119 | The contribution of the mouse to advances in human genetics |
| Q37122355 | The cystic fibrosis intestine |
| Q28512129 | The light peak of the electroretinogram is dependent on voltage-gated calcium channels and antagonized by bestrophin (best-1) |
| Q34106600 | The use of carboxymethylcellulose gel to increase non-viral gene transfer in mouse airways |
| Q27335978 | Trehalose biosynthesis promotes Pseudomonas aeruginosa pathogenicity in plants |
| Q36750909 | Ventilatory pattern and energy expenditure are altered in cystic fibrosis mice |
| Q79363097 | [Digestive symptoms in cystic fibrosis] |
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