Adult Cystic Fibrosis and Host Defense

Program Description

The Adult Cystic Fibrosis and Host Defense Research Program is directed by Dr. Joseph Pilewski. The clinical program is based in the Adult Cystic Fibrosis Clinic. Additional faculty members involved with this program include Dr. Michael Myerburg and Dr. Timothy Corcoran.

Dr. Pilewski’s research focuses on the pursuit of the pathogenesis of cystic fibrosis and the development of new drug and genetic therapies for this disease. His current research efforts are focused on the role of mucins in normal airway host defense and CF pathogenesis. The objectives of his work are to define the structure and function of the transmembrane mucin MUC1 in normal airway function, and to determine whether CF gene mutations alter the structure and function of MUC1. A second focus of research has been modulation of ion transport in normal and CF epithelia, with the goal of translational studies that seek to confirm in vitro observations and identify new therapies for CF. A third research focus has been the development of in vitro models of lung cancer for studies of immunotherapy and gene therapy for early lung cancer.  A variety of clinically relevant model systems, including primary airway epithelial cells derived from CF and non-CF lung transplant patients are being used to address this hypothesis.

Dr. Myerburg’s  primary research interests include understanding the role of the epithelial sodium channel, ENaC, in cystic fibrosis and other airway diseases.  The goal of this research is to delineate how ENaC activity is regulated and to develop novel therapeutic strategies to mitigate sodium hyperabsorption in the airway.  Additionally, he is interested in airway epithelial cell biology, as it relates to mucus clearance from the lung and host defense from inhaled pathogens.

Dr. Corcoran’s primary research interests include the development of aerosol medications and the study of aerosol drug delivery technology.  He is involved in the pre-clinical development of new aerosol medications and in clinical studies to measure deposited aerosol dose in the lung.   His other research interests include the assessment of clearance in the lung, and the development of therapies for cystic fibrosis and lung transplantation.

Program Facilities

This research program is currently located in the UPMC - Montefiore Building at the University of Pittsburgh. This program is a key component in the Center for Cystic Fibrosis Research under the direction of Dr. Raymond A. Frizzel.