Cell Biology Annual Report 2010-11 (FY 2011) - Department of Cell ...

Centers of the Department
Cystic Fibrosis Research Center
Cell Biology and Physiology
Annual Report
Center Director: Dr. Raymond A. Frizzell
The Cystic Fibrosis Foundation established a Research
Development Program Center for research in cystic fibrosis with
a five-year, $2 million grant in 1997. It was renewed in 2002 and
2007 and 2011. The primary goal of the Center is to focus the
attention of new and established investigators on multidisciplinary
approaches to improve the understanding and treatment of cystic fibrosis (CF), the most
common lethal genetic disease among Caucasians. In creating this Center, the CF Foundation
took advantage of unique opportunities present at the School of Medicine and the Children’s
Hospital at the University of Pittsburgh, including a large and accessible patient population for
pre-clinical and clinical research and excellent availability of patient lung tissue due to a large
volume of lung transplant activity. The University of Pittsburgh RDP Center is one of nine such
Centers supported by the CF Foundation in North America.
In addition to the RDP award, the Center was the recipient, in 2004 and 2010, of a Core Center
grant from the NIH (P30 entitled, “Basic and Clinical Studies of Cystic Fibrosis”). Three such
Centers were awarded nationally in the last funding round. The CF Research Center is directed
by Raymond A. Frizzell, Ph.D., with extensive interactions with clinical colleagues and
co-Directors, Drs. Joseph Pilewski and Jay Kolls. The NIH Center supports pilot research projects
and core facilities. The primary P30 award criterion was the presence of a significant research
base of existing extramural grants, awarded to Center investigators, to justify its Research Cores.
The current Center is a free-standing administrative unit and its primary cores are housed in the
Rangos Research Center at the Children’s Hospital of Pittsburgh, the Department of Cell Biology
and Physiology, and the Adult Pulmonary Division of the Department of Medicine.
The Center’s research efforts focus on several areas relevant to the understanding and treatment of
CF: basic studies of the function, protein interactions, trafficking and processing of the CF gene
product, CFTR and its disease-causing mutants; understanding the infection-inflammation issues
that compromise the function of CF airways; the development of new therapies and diagnostic
approaches for treating CF, and participation of Center investigators in clinical research. Our
funding mechanisms allow the Center to encourage interactions between investigators with longstanding
interests and accomplishments in CF research and to bring new investigators into the CF
field.
Research and Clinical Cores:
12
Human Airway Cell and Assays Core: This core provides access to patient materials obtained as a
result of lung transplant activities in the Department of Surgery. This core offers well
differentiated primary cultures of human bronchial epithelia to facilitate a variety of pre-clinical
research investigations. It has supplied cells to various academic and industrial investigators
involved in CF research. This core also provides functional assays of CFTR and other proteins.
Its assay menu includes fluorescence assays for anion permeability, transepithelial current,
conductance, impedance and current fluctuation analysis in polarized airway or other epithelial
cell cultures, both established cell lines and primary HBE cultures (above). Facilities and