The Cystic Fibrosis Research Center at the University of Pittsburgh currently has 55 members in eight departments and garners nearly $10 M in external grants and contracts to support its CF research efforts. These are focused in three major areas: The Center has a strong basic science component that addresses the Cell and Molecular Biology of CF, and is supported by NIH, NSF and Cystic Fibrosis Foundation (CFF) grants. Investigators in this group make extensive use of differentiated, primary human bronchial epithelia (HBE) for their work. They employ the methods of biochemistry, molecular biology, cell biology and electrophysiology to study CFTR and ENaC functions in epithelial membranes, how these pathways contribute to normal airway function, how they are trafficked between various compartments of the protein secretory and recycling pathways, and how pharmacological manipulation of channel biogenesis (correction) or channel activity (potentiation) influence HBE function. Studies of Lung Infection and Inflammation focus on the pulmonary inflammatory response to bacterial infection in HBE and animal models. These studies aim to improve our understanding of CF disease pathogenesis, define bio-markers for clinical trials, and identify targets for anti-inflammatory therapy. The Clinical Studies group translates basic findings into new therapies. It develops and evaluates methods to improve airway drug delivery and isotopic clearance assays that assess transmural airway liquid movements in vivo, to test therapeutics that target the core defect in CF. The proposed Research and Translational Core Center will be directed by Dr. Raymond Frizzell while Dr. Joseph Pilewski will serve as Associate Director. The Center is comprised of three scientific cores: Human Airway Cell and Assays (Frizzell, PI), Clinical Studies/Outcomes (Pilewski, PI), and Imaging (Watkins, PI). The Core Center will operate a Pilot and Feasibility Program to bring new investigators into CF research. This Center emphasizes the translation of basic knowledge into applied therapeutics.

Agency
National Institute of Health (NIH)
Type
Center Core Grants (P30)
Project #
5P30DK072506-10
Application #
8685245
Study Section
Special Emphasis Panel (ZDK1)
Program Officer
Eggerman, Thomas L
Project Start
Project End
Budget Start
Budget End
Support Year
10
Fiscal Year
2014
Total Cost
Indirect Cost
Name
University of Pittsburgh
Department
Anatomy/Cell Biology
Type
Schools of Medicine
DUNS #
City
Pittsburgh
State
PA
Country
United States
Zip Code
15213
Deslouches, Berthony; Hasek, Mary L; Craigo, Jodi K et al. (2016) Comparative functional properties of engineered cationic antimicrobial peptides consisting exclusively of tryptophan and either lysine or arginine. J Med Microbiol 65:554-65
Gong, Xiaoyan; Ahner, Annette; Roldan, Ariel et al. (2016) Non-native Conformers of Cystic Fibrosis Transmembrane Conductance Regulator NBD1 Are Recognized by Hsp27 and Conjugated to SUMO-2 for Degradation. J Biol Chem 291:2004-17
Melvin, Jeffrey A; Lashua, Lauren P; Kiedrowski, Megan R et al. (2016) Simultaneous Antibiofilm and Antiviral Activities of an Engineered Antimicrobial Peptide during Virus-Bacterium Coinfection. mSphere 1:
Joyce, Emily; Glasner, Paulina; Ranganathan, Sarangarajan et al. (2016) Tubulointerstitial nephritis: diagnosis, treatment, and monitoring. Pediatr Nephrol :
Salomon, Johanna J; Spahn, Stephan; Wang, Xiaohui et al. (2016) Generation and functional characterization of epithelial cells with stable expression of SLC26A9 Cl- channels. Am J Physiol Lung Cell Mol Physiol 310:L593-602
Ernst, Wayne L; Shome, Kuntala; Wu, Christine C et al. (2016) VAMP-associated Proteins (VAP) as Receptors That Couple Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Proteostasis with Lipid Homeostasis. J Biol Chem 291:5206-20
Shrestha, Archana; Hendricks, Matthew R; Bomberger, Jennifer M et al. (2016) Bystander Host Cell Killing Effects of Clostridium perfringens Enterotoxin. MBio 7:
Veit, Gudio; Avramescu, Radu G; Chiang, Annette N et al. (2016) From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations. Mol Biol Cell 27:424-33
Hendricks, Matthew R; Lashua, Lauren P; Fischer, Douglas K et al. (2016) Respiratory syncytial virus infection enhances Pseudomonas aeruginosa biofilm growth through dysregulation of nutritional immunity. Proc Natl Acad Sci U S A 113:1642-7
Melvin, Jeffrey A; Montelaro, Ronald C; Bomberger, Jennifer M (2016) Clinical potential of engineered cationic antimicrobial peptides against drug resistant biofilms. Expert Rev Anti Infect Ther 14:989-991

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