Published in Pediatr Res on June 01, 1978
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Defective beta adrenergic response of cystic fibrosis sweat glands in vivo and in vitro. J Clin Invest (1984) 2.17
Impaired beta adrenergic receptor binding and function in cystic fibrosis neutrophils. J Clin Invest (1981) 1.01
Beta adrenergic receptors in lymphocytes and granulocytes from patients with cystic fibrosis. J Clin Invest (1983) 0.98
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The yeast HML I silencer defines a heterochromatin domain boundary by directional establishment of silencing. Proc Natl Acad Sci U S A (1999) 1.48
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Dietary changes improve survival of CFTR S489X homozygous mutant mouse. Am J Physiol (1995) 1.34
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Early features of zidovudine-associated myopathy: histopathological findings and clinical correlations. Acta Neuropathol (1995) 1.26
Autonomic nervous system abnormalities and allergy. Ann Intern Med (1982) 1.22
Assisted ventilation for patients with cystic fibrosis. JAMA (1978) 1.20
Abnormal adrenergic and cholinergic sensitivity in cystic fibrosis. N Engl J Med (1980) 1.18
Metabolic abnormalities in skeletal muscle of patients receiving zidovudine therapy observed by 31P in vivo magnetic resonance spectroscopy. J Clin Invest (1995) 1.11
Function of the R domain in the cystic fibrosis transmembrane conductance regulator chloride channel. J Biol Chem (1997) 1.10
A single conductance pore for chloride ions formed by two cystic fibrosis transmembrane conductance regulator molecules. J Biol Chem (1999) 1.10
Gene transfer into the airway epithelium of animals by targeting the polymeric immunoglobulin receptor. J Clin Invest (1995) 1.08
Mortality and social class in New Zealand II: male mortality by major disease groupings. N Z Med J (1983) 1.07
Ibuprofen attenuates the inflammatory response to Pseudomonas aeruginosa in a rat model of chronic pulmonary infection. Implications for antiinflammatory therapy in cystic fibrosis. Am Rev Respir Dis (1990) 1.07
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A roentgenographic evaluation of frozen allografts versus autografts in anterior cervical spine fusions. Clin Orthop Relat Res (1976) 1.01
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Overexpression of R domain eliminates cAMP-stimulated Cl- secretion in 9/HTEo- cells in culture. Am J Physiol (1996) 0.98
Beta adrenergic receptors in lymphocytes and granulocytes from patients with cystic fibrosis. J Clin Invest (1983) 0.98
Slow conversions among subconductance states of cystic fibrosis transmembrane conductance regulator chloride channel. Biophys J (1996) 0.97
Intracellular loop between transmembrane segments IV and V of cystic fibrosis transmembrane conductance regulator is involved in regulation of chloride channel conductance state. J Biol Chem (1995) 0.97
Exercise intolerance due to a nonsense mutation in the mtDNA ND4 gene. Ann Neurol (1999) 0.97
Low sweat electrolytes in a patient with cystic fibrosis. Am J Med (1980) 0.96
Heterosexual behaviour and HIV risk in New Zealand: data from a national survey. Aust J Public Health (1995) 0.95
Sweat chloride concentration in adults with pulmonary diseases. Am Rev Respir Dis (1983) 0.95
Enhanced target gene knockdown by a bifunctional shRNA: a novel approach of RNA interference. Cancer Gene Ther (2010) 0.95
Bronchiectasis and oligospermia: two families. Thorax (1985) 0.94
Evolution of therapy for cystic fibrosis. N Engl J Med (1994) 0.94
Studies of cardiopulmonary blood volume. Measurement of left ventricular volume by dye dilution. Circulation (1967) 0.93
Terminal sialylation is altered in airway cells with impaired CFTR-mediated chloride transport. Am J Physiol Lung Cell Mol Physiol (2001) 0.92
Cortical MRI findings associated with rapid correction of hyponatremia. Neurology (2000) 0.92
Mortality and social class in New Zealand. III: male mortality by ethnic group. N Z Med J (1984) 0.91
Gene transfer into respiratory epithelial cells by targeting the polymeric immunoglobulin receptor. J Clin Invest (1993) 0.90
Sustained production of secretory component by human tracheal epithelial cells in primary culture. Am J Physiol (1991) 0.89
Autonomic and airway reactivity in obligate heterozygotes for cystic fibrosis. Am Rev Respir Dis (1984) 0.89
Conformation, independent of charge, in the R domain affects cystic fibrosis transmembrane conductance regulator channel openings. Biophys J (2000) 0.87
Pulmonary function in obligate heterozygotes for cystic fibrosis. Am Rev Respir Dis (1988) 0.87
Human epithelial cystic fibrosis transmembrane conductance regulator without exon 5 maintains partial chloride channel function in intracellular membranes. Biophys J (1996) 0.85
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Human fetal lung fibroblasts promote invasion of extracellular matrix by normal human tracheobronchial epithelial cells in vitro: a model of early airway gland development. Am J Respir Cell Mol Biol (1993) 0.85
The decline and fall of pulmonary function in cystic fibrosis: new models, new lessons. J Pediatr (1997) 0.84
Familial bronchiectasis. J Pediatr (1983) 0.84
What we know and what we do not know about cystic fibrosis transmembrane conductance regulator. Clin Chest Med (1998) 0.84
Isolated growth hormone deficiency and cystic fibrosis: a report of two cases. Am J Dis Child (1980) 0.83
Ligand substitution of receptor targeted DNA complexes affects gene transfer into hepatoma cells. Gene Ther (1998) 0.82
Comparison of psychomotor skills and amnesia after induction of anesthesia with midazolam or thiopental. Anesth Analg (1986) 0.82
The involvement of RNA in the initiation of DNA synthesis in mammalian cells. Artifacts arising during caesium-salt density-gradient centrifugation which simulate a covalent attachment of RNA to newly synthesized DNA. Eur J Biochem (1976) 0.82
In vitro transport of active alpha(1)-antitrypsin to the apical surface of epithelia by targeting the polymeric immunoglobulin receptor. Am J Respir Cell Mol Biol (1999) 0.81
Electrolytes and norepinephrine levels in blood of patients with cystic fibrosis. Clin Chim Acta (1979) 0.81
Autonomic nervous system abnormalities in cystic fibrosis. J Chronic Dis (1983) 0.81
Adenylate cyclase in leukocytes from patients with cystic fibrosis. J Lab Clin Med (1980) 0.81
Hormone-stimulated cyclic AMP production by skin fibroblasts cultured from healthy persons and patients with cystic fibrosis. Pediatr Res (1980) 0.81
Cystic fibrosis from bench to bedside. N Engl J Med (1991) 0.80
Gene transfer into hepatoma cell lines via the serpin enzyme complex receptor. Am J Physiol (1997) 0.80
Beta-adrenergic responses in drug-free subjects with asthma. J Allergy Clin Immunol (1986) 0.80
Defining strategies to extend duration of gene expression from targeted compacted DNA vectors. Gene Ther (2004) 0.80
The impact of chemokine receptor CX3CR1 deficiency during respiratory infections with Mycobacterium tuberculosis or Francisella tularensis. Clin Exp Immunol (2009) 0.79
Distribution of retinol-binding protein in tissues. Vitam Horm (1974) 0.79
Immunologic responses to gene transfer into mice via the polymeric immunoglobulin receptor. Gene Ther (1996) 0.79
Chain length of the polylysine in receptor-targeted gene transfer complexes affects duration of reporter gene expression both in vitro and in vivo. J Biol Chem (1999) 0.79
Single chain Fv: a ligand in receptor-mediated gene delivery. Gene Ther (2001) 0.79
Adenosine 3:5' cyclic monophosphate synthesis by human tracheal epithelial cells. Am J Respir Cell Mol Biol (1990) 0.79
Possible hazard of timed-release aspirin in a patient with pancreatic insufficiency. Arch Intern Med (1979) 0.78
Species differentiation and antibiotic susceptibility testing with DNA microarrays. J Appl Microbiol (2004) 0.78
Resistance of human tracheal epithelial cells to killing by neutrophils, neutrophil elastase, and Pseudomonas elastase. Am J Respir Cell Mol Biol (1993) 0.78
Human tracheobronchial epithelial cells direct migration of lung fibroblasts in three-dimensional collagen gels. Am J Physiol (1992) 0.78
Normal alpha 2-adrenergic responses in platelets from patients with asthma. J Allergy Clin Immunol (1982) 0.77
Progression of pulmonary disease after disappearance of Pseudomonas in cystic fibrosis. Am J Respir Crit Care Med (1995) 0.77
The influence of practitioner and practice characteristics on service activity levels in a New Zealand general practice sample. Community Health Stud (1989) 0.77
Growth in children with chronic lung disease. N Engl J Med (2000) 0.77
Rectification of cystic fibrosis transmembrane conductance regulator chloride channel mediated by extracellular divalent cations. Biophys J (1996) 0.76
Physiologic implications of the autonomic aberrations in cystic fibrosis. Horm Metab Res (1986) 0.76
Pupillary responses and airway reactivity in asthma. J Allergy Clin Immunol (1986) 0.76
Lymphocyte and granulocyte phosphatidylethanolamine N-methyltransferase: properties and activity in cystic fibrosis. Pediatr Res (1986) 0.76
Pathophysiology of cystic fibrosis with emphasis on salivary gland involvement. J Dent Res (1987) 0.76
Chronic hypoxia increases beta-adrenergic receptor density in the lungs of young and old rats. Mech Ageing Dev (1991) 0.76
Effect of age on acute regulation of beta-adrenergic responses in mononuclear leukocytes. J Gerontol (1989) 0.75
Ferret tracheal epithelial cells grown in vitro are resistant to lethal injury by activated neutrophils. Am J Respir Cell Mol Biol (1991) 0.75
Saturday's children. Am J Respir Crit Care Med (1995) 0.75
Neurology of human immunodeficiency virus infection--past, present, and future. West J Med (1995) 0.75
Interest of freshman medical students in family practice. J S C Med Assoc (1971) 0.75