Richard Boucher, M.D., is the Kenan Professor of Medicine and the Director of Cystic Fibrosis and Pulmonary Diseases Research and Treatment Center at the University of North Carolina at Chapel Hill.
Dr. Boucher received his medical degree from Columbia University and completed his residency at the Royal Victoria Hospital in Montreal, Canada. He joined the University of North Carolina at Chapel Hill School of Medicine faculty in 1977.
Dr. Boucher has directed the Cystic Fibrosis and Pulmonary Diseases Research and Treatment Center since 1988. He is also the co-director of the UNC Gene Therapy Center and the Division of Pulmonary & Critical Care Medicine.
Dr. Boucher has published more than 300 articles and has received numerous awards for his research on Cystic Fibrosis and pulmonary diseases, including the Distinguished Scientific Achievement Award from the American Thoracic Society and 1st Annual Champion for a Cure Award from the Cystic Fibrosis Foundation. In 1997, he was named the William Rand Kenan Professor in the department of medicine.
Boucher is the principal investigator of two Program Project Grants and one Specialized Center of Research Grant from the National Institutes of Health. The Boucher lab continues its major interest in the functions of airway epithelia in health and disease. Clinical studies include trials of novel drugs and gene transfer vectors for cystic fibrosis and mechanisms of exacerbations in chronic bronchitis.
Industry Expertise (3)
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Columbia University: M.D. Degree, Internal Medicine
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Aussie Scientists’ New X-ray Imaging System Helps Monitor Cystic Fibrosis Treatment Effectiveness
Cystic Fibrosis News Today online
Group research conducted during 2012 was centered on developing novel synchrotron based techniques for assessing airways physiological function. A collaborative project with physicists from Monash University and the Australian Synchrotron was established to develop novel X-ray imaging approaches effective in living mouse airways, which was reported in an Open Access PLOS 1 paper published last year entitled: “Measuring Airway Surface Liquid Depth in Ex Vivo Mouse Airways by X-Ray Imaging for the Assessment of Cystic Fibrosis Airway Therapies”...
UNC Researchers Find New CF Drugs Unsuspectedly Counteract One Another’s Effectiveness
Cystic Fibrosis News Today online
Findings of new study led by Martina Gentzsch, PhD, at the University of North Carolina School of Medicine and the UNC Marsico Lung Institute in Chapel Hill, N.C. could help drug developers improve compounds designed to correct CFTR proteins in cystic fibrosis (CF) patients. In lab experiments using tissue samples cultured from cystic fibrosis patients, UNC Dr. Gentzsch and her team of scientists have demonstrated that a new CF drug counteracts the intended beneficial molecular effect of another CF drug...
Smoking cigarettes simulates cystic fibrosis
If you smoke cigarettes, you have more in common with someone who has cystic fibrosis than you think. A new research report appearing online in the FASEB Journal (http://www.fasebj.org) shows that smoking cigarettes affects the lungs in a way that is very similar to cystic fibrosis, a life threatening disease affecting the lungs and other organs...
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene result in defective epithelial cAMP-dependent Cl- secretion and increased airway Na+ absorption. The mechanistic links between these altered ion transport processes and the pathogenesis of cystic fibrosis lung disease, however, are unclear. To test the hypothesis that accelerated Na+ transport alone can produce cystic fibrosis-like lung disease, we generated mice with airway-specific overexpression of epithelial Na+ channels (ENaC). Here we show that increased airway Na+ absorption in vivo caused airway surface liquid (ASL) volume depletion, increased mucus concentration, delayed mucus transport and mucus adhesion to airway surfaces. Defective mucus transport caused a severe spontaneous lung disease sharing features with cystic fibrosis, including mucus obstruction, goblet cell metaplasia, neutrophilic inflammation and poor bacterial clearance. We conclude that increasing airway Na+ absorption initiates cystic fibrosis-like lung disease and produces a model for the study of the pathogenesis and therapy of this disease.
Cystic fibrosis (CF) is characterized by defective mucociliary clearance and chronic airway infection by a complex microbiota. Infection, persistent inflammation and periodic episodes of acute pulmonary exacerbation contribute to an irreversible decline in CF lung function. While the factors leading to acute exacerbations are poorly understood, antibiotic treatment can temporarily resolve pulmonary symptoms and partially restore lung function. Previous studies indicated that exacerbations may be associated with changes in microbial densities and the acquisition of new microbial species. Given the complexity of the CF microbiota, we applied massively parallel pyrosequencing to identify changes in airway microbial community structure in 23 adult CF patients during acute pulmonary exacerbation, after antibiotic treatment and during periods of stable disease.
Aquaporins (AQPs) facilitate water transport across epithelia and play an important role in normal physiology and disease in the human airways. We used in situ hybridization and immunofluorescence to determine the expression and cellular localization of AQPs 5, 4, and 3 in human airway sections. In nose and bronchial epithelia, AQP5 is expressed at the apical membrane of columnar cells of the superficial epithelium and submucosal gland acinar cells. AQP4 was detected in basolateral membranes in ciliated ducts and by in situ in gland acinar cells. AQP3 is present on basal cells of both superficial epithelium and gland acinus. In these regions AQPs 5, 4, and 3 are appropriately situated to permit transepithelial water permeability. In the small airways (proximal and terminal bronchioles) AQP3 distribution shifts from basal cell to surface expression (i.e., localized to the apical membrane of proximal and terminal bronchioles) and is the only AQP identified in this region of the human lung. The alveolar epithelium has all three AQPs represented, with AQP5 and AQP4 localized to type I pneumocytes and AQP3 to type II cells. This study describes an intricate network of AQP expression that mediates water transport across the human airway epithelium.
The pathogenesis of cystic fibrosis (CF) airways infection is unknown. Two hypotheses, “hypotonic [low salt]/defensin” and “isotonic volume transport/mucus clearance,” attempt to link defects in cystic fibrosis transmembrane conductance regulator–mediated ion transport to CF airways disease. We tested these hypotheses with planar and cylindrical culture models and found no evidence that the liquids lining airway surfaces were hypotonic or that salt concentrations differed between CF and normal cultures. In contrast, CF airway epithelia exhibited abnormally high rates of airway surface liquid absorption, which depleted the periciliary liquid layer and abolished mucus transport. The failure to clear thickened mucus from airway surfaces likely initiates CF airways infection. These data indicate that therapy for CF lung disease should not be directed at modulation of ionic composition, but rather at restoring volume (salt and water) on airway surfaces.
Cystic fibrosis results from defects in the gene encoding a cyclic adenosine monophosphate-dependent chloride ion channel known as the cystic fibrosis transmembrane conductance regulator (CFTR). To create an animal model for cystic fibrosis, mice were generated from embryonic stem cells in which the CFTR gene was disrupted by gene targeting. Mice homozygous for the disrupted gene display many features common to young human cystic fibrosis patients, including failure to thrive, meconium ileus, alteration of mucous and serous glands, and obstruction of glandlike structures with inspissated eosinophilic material. Death resulting from intestinal obstruction usually occurs before 40 days of age.