The UAB P30 Research and Translation Core Center consolidates a large number of externally funded cystic fibrosis (CF) research programs on our campus. During the first funding period, the P30 has made important contributions to multiple UAB laboratories pursuing research relevant to CFTR cellular and Structural biology and CF pathogenesis. By virtue of the NIH Center, translational research at our Institution has advanced significantly in the past four years The richness of CF basic science at UAB has grown in parallel with this translational expansion. The P30 Center has allowed investigators at UAB and collaborating sites to improve understanding of cystic fibrosis disease mechanism and furnished novel opportunities to aggressively apply this Information towards experimental therapeutics. This NIH Center includes three Scientific Cores that help organize efforts of CF faculty towards the common and essential goal of helping Individuals with CF. The Cores include: Core A: Cell Model and Assay Core (KL Kirk, PI);Core B: Animal Models Core (DM Bedwell, PI);and Core C: Clinical and Translational Core (SM Rowe, PI). Each Core provides leading-edge assays, specialized reagents and valved expertise. The P30 has also engaged new investigators through a Pilot and Feasibility mechanism integral to Center vitality. In addition to providing a platform from which junior and senior scientists are brought into the field, Pilot Projects serve as a means of rapidly testing exciting advances, particularly from the perspective of clinical translation. Two Pilot and Feasibility Projects are proposed: Project 1: SG Aller, PI. """"""""Structure-based correction of the major defect in cystic fibrosis"""""""";and Project 2: GA Caldwell, PI. """"""""Investigation of torsinA modulation as a therapeutic strategy for cystic fibrosis"""""""". Through these scientific initiatives, th P30 has enhanced a collaborative environment for cystic fibrosis research at UAB, and is well positioned to continue in this capacity in the future.

Public Health Relevance

The UAB P30 embodies a well funded Research Base dedicated to expanding the boundaries of knowledge regarding cystic fibrosis pathogenesis and therapy. Progress is measured by innovative contributions and scientific discovery designed to improve the lives of patients with the disease.

Agency
National Institute of Health (NIH)
Institute
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Type
Center Core Grants (P30)
Project #
5P30DK072482-08
Application #
8685239
Study Section
Special Emphasis Panel (ZDK1-GRB-7 (J1))
Program Officer
Eggerman, Thomas L
Project Start
2005-07-01
Project End
2017-04-30
Budget Start
2014-05-01
Budget End
2015-04-30
Support Year
8
Fiscal Year
2014
Total Cost
$1,087,084
Indirect Cost
$312,910
Name
University of Alabama Birmingham
Department
Internal Medicine/Medicine
Type
Schools of Medicine
DUNS #
063690705
City
Birmingham
State
AL
Country
United States
Zip Code
35294
Wang, Wei; Hong, Jeong S; Rab, Andras et al. (2016) Robust Stimulation of W1282X-CFTR Channel Activity by a Combination of Allosteric Modulators. PLoS One 11:e0152232
Mutyam, Venkateshwar; Libby, Emily Falk; Peng, Ning et al. (2016) Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation. J Cyst Fibros :
Wei, Shipeng; Roessler, Bryan C; Icyuz, Mert et al. (2016) Long-range coupling between the extracellular gates and the intracellular ATP binding domains of multidrug resistance protein pumps and cystic fibrosis transmembrane conductance regulator channels. FASEB J 30:1247-62
Ehrhardt, Annette; Chung, W Joon; Pyle, Louise C et al. (2016) Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop. J Biol Chem 291:1854-65
Roy, Bijoyita; Friesen, Westley J; Tomizawa, Yuki et al. (2016) Ataluren stimulates ribosomal selection of near-cognate tRNAs to promote nonsense suppression. Proc Natl Acad Sci U S A 113:12508-12513
Mutyam, Venkateshwar; Du, Ming; Xue, Xiaojiao et al. (2016) Discovery of Clinically Approved Agents That Promote Suppression of Cystic Fibrosis Transmembrane Conductance Regulator Nonsense Mutations. Am J Respir Crit Care Med 194:1092-1103
Bali, Vedrana; Lazrak, Ahmed; Guroji, Purushotham et al. (2016) Mechanistic Approaches to Improve Correction of the Most Common Disease-Causing Mutation in Cystic Fibrosis. PLoS One 11:e0155882
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
Solomon, George M; Raju, S Vamsee; Dransfield, Mark T et al. (2016) Therapeutic Approaches to Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction in Chronic Bronchitis. Ann Am Thorac Soc 13 Suppl 2:S169-76
Bali, Vedrana; Lazrak, Ahmed; Guroji, Purushotham et al. (2016) A synonymous codon change alters the drug sensitivity of ΔF508 cystic fibrosis transmembrane conductance regulator. FASEB J 30:201-13

Showing the most recent 10 out of 117 publications