John P Clancy

Summary

Affiliation: University of Alabama at Birmingham
Country: USA

Publications

  1. ncbi request reprint Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis
    J P Clancy
    Department of Pediatrics, University of Alabama at Birmingham, USA
    Am J Respir Crit Care Med 163:1683-92. 2001
  2. pmc Restoration of W1282X CFTR activity by enhanced expression
    Steven M Rowe
    Department of Medicine, Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, Alabama 35294 0006, USA
    Am J Respir Cell Mol Biol 37:347-56. 2007
  3. ncbi request reprint Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial
    Richard B Moss
    Department of Pediatrics, Stanford University, Stanford, CA 94305, and Division of Respiratory Diseases, Children s Hospital Boston, MA 02115, USA
    Hum Gene Ther 18:726-32. 2007
  4. pmc No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations
    John P Clancy
    Department of Pediatrics, University of Alabama at Birmingham, 1600 7th Avenue South, Birmingham, AL 35233, USA
    Am J Respir Cell Mol Biol 37:57-66. 2007
  5. pmc Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials
    Steven M Rowe
    Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama 35294 0006, USA
    Proc Am Thorac Soc 4:387-98. 2007
  6. doi request reprint Pharmaceuticals targeting nonsense mutations in genetic diseases: progress in development
    Steven M Rowe
    Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35233, USA
    BioDrugs 23:165-74. 2009
  7. doi request reprint Ivacaftor treatment of cystic fibrosis patients with the G551D mutation: a review of the evidence
    Kavitha Kotha
    Division of Pulmonary Medicine, Department of Pediatrics, Cincinnati Children s Hospital Medical Center and the University of Cincinnati, Cincinnati, OH, USA
    Ther Adv Respir Dis 7:288-96. 2013
  8. pmc Reduced sodium transport with nasal administration of the prostasin inhibitor camostat in subjects with cystic fibrosis
    Steven M Rowe
    Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35294 0006, USA
    Chest 144:200-7. 2013
  9. pmc Progress in cystic fibrosis and the CF Therapeutics Development Network
    Steven M Rowe
    Department of Medicine, University of Alabama at Birmingham, 1819 University Boulevard MCLM 768, Birmingham, AL 35294, USA
    Thorax 67:882-90. 2012
  10. ncbi request reprint Mutations in the amino terminus of the cystic fibrosis transmembrane conductance regulator enhance endocytosis
    Asta Jurkuvenaite
    Department of Cell Biology, University of Alabama at Birmingham, AL 35294, USA
    J Biol Chem 281:3329-34. 2006

Collaborators

Detail Information

Publications20

  1. ncbi request reprint Evidence that systemic gentamicin suppresses premature stop mutations in patients with cystic fibrosis
    J P Clancy
    Department of Pediatrics, University of Alabama at Birmingham, USA
    Am J Respir Crit Care Med 163:1683-92. 2001
    ..Together, these results suggest that gentamicin treatment can suppress premature stop mutations in airway cells from patients with CF, and produce small increases in CFTR Cl(-) conductance (as measured by the nasal PD) in vivo...
  2. pmc Restoration of W1282X CFTR activity by enhanced expression
    Steven M Rowe
    Department of Medicine, Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, Birmingham, Alabama 35294 0006, USA
    Am J Respir Cell Mol Biol 37:347-56. 2007
    ..These findings provide evidence that W1282X CFTR exhibits membrane localization and retained chloride channel function after enhanced expression, and suggest that patients harboring this mutation may be more susceptible to CFTR rescue...
  3. ncbi request reprint Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial
    Richard B Moss
    Department of Pediatrics, Stanford University, Stanford, CA 94305, and Division of Respiratory Diseases, Children s Hospital Boston, MA 02115, USA
    Hum Gene Ther 18:726-32. 2007
    ..Because gene transfer is the simplest, most basic way to correct the underlying genetic defect that leads to disease in CF, further research is warranted to develop an effective gene transfer agent for the treatment of CF...
  4. pmc No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations
    John P Clancy
    Department of Pediatrics, University of Alabama at Birmingham, 1600 7th Avenue South, Birmingham, AL 35233, USA
    Am J Respir Cell Mol Biol 37:57-66. 2007
    ..The study provides valuable information on parameters of the nasal potential difference measurements for use in future multicenter clinical trials...
  5. pmc Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials
    Steven M Rowe
    Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama 35294 0006, USA
    Proc Am Thorac Soc 4:387-98. 2007
    ..Recommendations are presented to advance our understanding of these biomarkers and to improve their capacity to predict cystic fibrosis outcomes...
  6. doi request reprint Pharmaceuticals targeting nonsense mutations in genetic diseases: progress in development
    Steven M Rowe
    Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35233, USA
    BioDrugs 23:165-74. 2009
    ..These results, coupled with our improved understanding of how translation termination is regulated at PTCs, will help guide future directions of research involving this innovative treatment strategy for genetic diseases...
  7. doi request reprint Ivacaftor treatment of cystic fibrosis patients with the G551D mutation: a review of the evidence
    Kavitha Kotha
    Division of Pulmonary Medicine, Department of Pediatrics, Cincinnati Children s Hospital Medical Center and the University of Cincinnati, Cincinnati, OH, USA
    Ther Adv Respir Dis 7:288-96. 2013
    ....
  8. pmc Reduced sodium transport with nasal administration of the prostasin inhibitor camostat in subjects with cystic fibrosis
    Steven M Rowe
    Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35294 0006, USA
    Chest 144:200-7. 2013
    ..Its direct inhibition by camostat represents a potential approach to inhibiting sodium transport in cystic fibrosis (CF)...
  9. pmc Progress in cystic fibrosis and the CF Therapeutics Development Network
    Steven M Rowe
    Department of Medicine, University of Alabama at Birmingham, 1819 University Boulevard MCLM 768, Birmingham, AL 35294, USA
    Thorax 67:882-90. 2012
    ..Efforts to advance CF biomarkers, necessary to accelerate the therapeutic goals of the network, are also summarised...
  10. ncbi request reprint Mutations in the amino terminus of the cystic fibrosis transmembrane conductance regulator enhance endocytosis
    Asta Jurkuvenaite
    Department of Cell Biology, University of Alabama at Birmingham, AL 35294, USA
    J Biol Chem 281:3329-34. 2006
    ..These two additive effects contribute to the loss of surface expression and the associated defect in chloride conductance that is consistent with a disease phenotype...
  11. ncbi request reprint Advances in cystic fibrosis therapies
    Steven M Rowe
    Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35294, USA
    Curr Opin Pediatr 18:604-13. 2006
    ..This review describes recent developments in cystic fibrosis pulmonary therapies intended to treat various facets of the disease, including several treatments currently in development...
  12. pmc Nucleosides accelerate inflammatory osteolysis, acting as distinct innate immune activators
    George Pan
    Department of Pediatrics, Emory University, Atlanta, GA 30322, USA
    J Bone Miner Res 26:1913-25. 2011
    ..These observations provide new insight into the pathogenesis and underlying mechanism of bone destruction in inflammatory autoimmune osteoarthritis...
  13. ncbi request reprint Repeated adeno-associated virus serotype 2 aerosol-mediated cystic fibrosis transmembrane regulator gene transfer to the lungs of patients with cystic fibrosis: a multicenter, double-blind, placebo-controlled trial
    Richard B Moss
    Department of Pediatrics, Stanford University, Stanford, CA, USA
    Chest 125:509-21. 2004
    ....
  14. ncbi request reprint Adenosine receptors and phosphodiesterase inhibitors stimulate Cl- secretion in Calu-3 cells
    Bryan R Cobb
    Department of Human Genetics, Gregory Fleming James Cystic Fibrosis Research Center, University of Alabama at Birmingham, 35233, USA
    Am J Respir Cell Mol Biol 29:410-8. 2003
    ..These studies also indicate that PDEis have the capacity to augment an endogenous CFTR-activating pathway in an "in vivo"-like model system, and supports future investigations of these agents relevant to cystic fibrosis...
  15. pmc Extracorporeal membrane oxygenation causes loss of intestinal epithelial barrier in the newborn piglet
    Ashish R Kurundkar
    Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL 35294, USA
    Pediatr Res 68:128-33. 2010
    ..On the basis of these data, we conclude that ECMO is an independent cause of gut barrier dysfunction and bacterial translocation may be an important contributor to ECMO-related inflammation...
  16. ncbi request reprint Up-regulation of AMP-activated kinase by dysfunctional cystic fibrosis transmembrane conductance regulator in cystic fibrosis airway epithelial cells mitigates excessive inflammation
    Kenneth R Hallows
    Department of Medicine, University of Pittsburgh School of Medicine, Pennsylvania 15261, USA
    J Biol Chem 281:4231-41. 2006
    ..We propose that therapies to activate AMPK in the CF airway may be beneficial in reducing excessive airway inflammation, a major cause of CF morbidity...
  17. pmc A macromolecular complex of beta 2 adrenergic receptor, CFTR, and ezrin/radixin/moesin-binding phosphoprotein 50 is regulated by PKA
    Anjaparavanda P Naren
    Department of Physiology and Biophysics, Medical Center, University of Tennessee Health Science Center, Memphis 38163, USA
    Proc Natl Acad Sci U S A 100:342-6. 2003
    ..Deleting the regulatory domain of CFTR abolishes PKA regulation of complex assembly. This report summarizes a macromolecular signaling complex involving CFTR, the implications of which may be relevant to CFTR-dysfunction diseases...
  18. ncbi request reprint Assessment of CFTR localisation in native airway epithelial cells obtained by nasal brushing
    Ceinwen M Harris
    Department of Medical Biochemistry and Immunology, University of Wales College of Medicine, Heath Park, Cardiff, Wales CF14 4XN, UK
    J Cyst Fibros 3:43-8. 2004
    ..The protocols are a consensus, following comparison of some aspects of methods currently used in the authors' laboratories...
  19. ncbi request reprint Activation of chloride secretion in cystic fibrosis cells and tissues by the substituted imidazole SRI 2931
    Julie R Jones
    Vanderbilt University Medical Center, Department of Molecular Physiology and Biophysics, Nashville, Tennessee, USA
    Biochemistry 42:13241-9. 2003
    ..The magnitude, longevity, and mechanism of action of the response provide a tool for dissecting pathways of epithelial ATP extracellular signaling and Cl(-) permeability...
  20. ncbi request reprint The Caregiver Quality of Life Cystic Fibrosis (CQOLCF) scale: modification and validation of an instrument to measure quality of life in cystic fibrosis family caregivers
    Whitney Boling
    Department of Health and Human Performance, University of Houston, TX 77204 6015, USA
    Qual Life Res 12:1119-26. 2003
    ..Qual Life Res 2000; 8: 55-63) as the Caregiver Quality of Life Cystic Fibrosis (CQOLCF) scale, validate it with cystic fibrosis (CF) family caregivers, and assess caregiver quality of life QOL with patient disease severity...