Marsico Lung Institute/UNC Cystic Fibrosis Center

CFTR protein expression in well-differentiated human bronchial epithelial cultures
CFTR protein expression in well-differentiated human bronchial epithelial cultures. Well-differentiated cultures derived from human bronchial epithelial tissues were immunostained with CFTR and tubulin antibodies and analyzed on a Leica SP2 laser confocal microscope. The image represents an overlay of the DIC (grayscale), CFTR (red), cilia (tubulin, green), and nuclei (DAPI, blue) confocal planes, and depicts an epithelial cell sheet that contains a group of ciliated cells surrounding a goblet cell (bottle-shaped cell with no cilia). CFTR is expressed only at the apical membrane of ciliated cells, but not goblet cells. Magnification x190. Reproduced from Kreda et al 2005 Mol Biol Cell 16, 2154 with permission of ASCB / MBC.

Introduction

By Richard C. Boucher, MD, Director

The Cystic Fibrosis and Pulmonary Diseases Research and Treatment Center (CF Center) at the University of North Carolina at Chapel Hill traditionally has had a single goal: to “cure” CF lung disease. To this end, we assembled a broad-based biomedical research team to tackle this goal. Thus, we have wide-ranging skills in ion transport physiology (Ussing chamber, patch clamp, confocal surface liquid imaging), mucus cell/mucin secretion biology and biochemistry, transgenic and gene-targeted mouse models, in vivo measures of mucociliary clearance and measures of mucin concentration/hydration, and Phase I clinical trials. In addition, we have added expertise focused on specific CF microbiologic problems of the lung, including Pseudomonas aeruginosa, anaerobic bacteria, and now Burkholderia cepacia. These efforts are complemented by a major effort to identify genetic modifiers of all facets of CF airways pathogenesis. Thus, the airways pathobiology/therapeutics efforts have produced multiple collaborations within the UNC MLI, multiple national/international collaborations, and, indeed, collaboration with the CFF Mucus Consortium and Gene Modifier groups.

Read the rest of Dr. Boucher’s Introduction here.