PubRank

J P Clancy

Author PubWeight™ 52.48‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med 2010 6.23
2 Recombinant human acid [alpha]-glucosidase: major clinical benefits in infantile-onset Pompe disease. Neurology 2006 5.67
3 A novel proteolytic cascade generates an extracellular matrix-derived chemoattractant in chronic neutrophilic inflammation. J Immunol 2008 2.58
4 Regulation of chloride channels by protein kinase C in normal and cystic fibrosis airway epithelia. Science 1989 2.46
5 Suppression of a CFTR premature stop mutation in a bronchial epithelial cell line. Nat Med 1997 2.24
6 Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers. J Physiol 2005 1.97
7 Cigarette smoke induces systemic defects in cystic fibrosis transmembrane conductance regulator function. Am J Respir Crit Care Med 2013 1.93
8 Potential role of high-mobility group box 1 in cystic fibrosis airway disease. Am J Respir Crit Care Med 2008 1.62
9 Endorectal repair of rectocele revisited. Br J Surg 1997 1.57
10 A(2) adenosine receptors regulate CFTR through PKA and PLA(2). Am J Physiol Lung Cell Mol Physiol 2002 1.51
11 Effects of megestrol acetate on weight gain, body composition, and pulmonary function in patients with cystic fibrosis. J Pediatr 2002 1.44
12 Matrix metalloprotease-9 dysregulation in lower airway secretions of cystic fibrosis patients. Am J Physiol Lung Cell Mol Physiol 2007 1.41
13 Cystic fibrosis transmembrane conductance regulator facilitates ATP release by stimulating a separate ATP release channel for autocrine control of cell volume regulation. J Biol Chem 2000 1.40
14 Efficient endocytosis of the cystic fibrosis transmembrane conductance regulator requires a tyrosine-based signal. J Biol Chem 1999 1.35
15 Activation of airway cl- secretion in human subjects by adenosine. Am J Respir Cell Mol Biol 2004 1.32
16 DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers. Pulm Pharmacol Ther 2010 1.16
17 Extracellular zinc and ATP restore chloride secretion across cystic fibrosis airway epithelia by triggering calcium entry. J Biol Chem 2003 1.12
18 Activation of the cystic fibrosis transmembrane conductance regulator by the flavonoid quercetin: potential use as a biomarker of ΔF508 cystic fibrosis transmembrane conductance regulator rescue. Am J Respir Cell Mol Biol 2009 1.07
19 Genetic and reproductive knowledge among adolescents and adults with cystic fibrosis. Chest 2008 1.07
20 A clinical inflammatory syndrome attributable to aerosolized lipid-DNA administration in cystic fibrosis. Hum Gene Ther 2001 1.04
21 Matrix metalloproteinase activity in pediatric acute lung injury. Int J Med Sci 2008 1.01
22 Plasma TGF-β₁ in pediatric cystic fibrosis: potential biomarker of lung disease and response to therapy. Pediatr Pulmonol 2011 0.98
23 Myofibroblast differentiation and enhanced TGF-B signaling in cystic fibrosis lung disease. PLoS One 2013 0.95
24 Relationship between airway ion transport and a mild pulmonary disease mutation in CFTR. Am J Respir Crit Care Med 1997 0.94
25 Early elevation of matrix metalloproteinase-8 and -9 in pediatric ARDS is associated with an increased risk of prolonged mechanical ventilation. PLoS One 2011 0.94
26 Adenosine regulation of cystic fibrosis transmembrane conductance regulator through prostenoids in airway epithelia. Am J Respir Cell Mol Biol 2006 0.93
27 Adenosine receptors, cystic fibrosis, and airway hydration. Handb Exp Pharmacol 2009 0.92
28 Ivacaftor: the first therapy acting on the primary cause of cystic fibrosis. Drugs Today (Barc) 2013 0.91
29 Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators. Am J Physiol Lung Cell Mol Physiol 2011 0.91
30 Spin waves and quantum criticality in the frustrated XY pyrochlore antiferromagnet Er2Ti2O7. Phys Rev Lett 2008 0.88
31 Structural Fluctuations in the spin-liquid state of Tb2Ti2O7. Phys Rev Lett 2007 0.85
32 A truncated CFTR protein rescues endogenous DeltaF508-CFTR and corrects chloride transport in mice. FASEB J 2009 0.84
33 A breath of fresh air. Sci Am 2011 0.81
34 A method for the rapid detection of recombinant CFTR during gene therapy in cystic fibrosis. Gene Ther 1996 0.77
35 Diagnosing cystic fibrosis in patients with non-diagnostic results: the case for intestinal current measurements. Thorax 2010 0.75
36 Field-induced order and spin waves in the pyrochlore antiferromagnet Tb2Ti207. Phys Rev Lett 2006 0.75
37 Membrane transplantation to correct integral membrane protein defects. J Mol Med (Berl) 2003 0.75
38 Magnetoelastics of a spin liquid: X-ray diffraction studies of Tb2Ti2O7 in pulsed magnetic fields. Phys Rev Lett 2010 0.75
39 Phosphorylation-dependent regulation of apical membrane chloride channels in normal and cystic fibrosis airway epithelium. Ann N Y Acad Sci 1989 0.75