Public Health Relevance

The proposed studies are specifically relevant to cystic fibrosis as they focus on a here-to-for neglected fundamental aspect of the molecular defect. As CFTR is also sensitive to environmental insults of the lung that contribute to COPD, the project is also relevant to that major health problem. Even more broadly, CFTR is also important in the functioning of the intestinal and reproductive tracts and other tissues such as the sweat gland, important in body temperature control.

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Research Program Projects (P01)
Project #
5P01HL110873-03
Application #
8658456
Study Section
Heart, Lung, and Blood Initial Review Group (HLBP)
Project Start
Project End
Budget Start
2014-05-01
Budget End
2015-04-30
Support Year
3
Fiscal Year
2014
Total Cost
$360,966
Indirect Cost
$115,996
Name
University of North Carolina Chapel Hill
Department
Type
DUNS #
608195277
City
Chapel Hill
State
NC
Country
United States
Zip Code
27599
He, Lihua; Aleksandrov, Andrei A; An, Jianli et al. (2015) Restoration of NBD1 thermal stability is necessary and sufficient to correct ?F508 CFTR folding and assembly. J Mol Biol 427:106-20
Roy, Michelle G; Livraghi-Butrico, Alessandra; Fletcher, Ashley A et al. (2014) Muc5b is required for airway defence. Nature 505:412-6
Henderson, Ashley G; Ehre, Camille; Button, Brian et al. (2014) Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure. J Clin Invest 124:3047-60
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Donnelley, Martin; Morgan, Kaye S; Siu, Karen K W et al. (2014) Non-invasive airway health assessment: synchrotron imaging reveals effects of rehydrating treatments on mucociliary transit in-vivo. Sci Rep 4:3689
Cholon, Deborah M; Quinney, Nancy L; Fulcher, M Leslie et al. (2014) Potentiator ivacaftor abrogates pharmacological correction of ?F508 CFTR in cystic fibrosis. Sci Transl Med 6:246ra96
Bove, Peter F; Dang, Hong; Cheluvaraju, Chaitra et al. (2014) Breaking the in vitro alveolar type II cell proliferation barrier while retaining ion transport properties. Am J Respir Cell Mol Biol 50:767-76
Guo, Xueliang; Zheng, Shuo; Dang, Hong et al. (2014) Genome reference and sequence variation in the large repetitive central exon of human MUC5AC. Am J Respir Cell Mol Biol 50:223-32
Yang, Zhengrong; Wang, Chi; Zhou, Qingxian et al. (2014) Membrane protein stability can be compromised by detergent interactions with the extramembranous soluble domains. Protein Sci 23:769-89
Hill, David B; Vasquez, Paula A; Mellnik, John et al. (2014) A biophysical basis for mucus solids concentration as a candidate biomarker for airways disease. PLoS One 9:e87681

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