Abstract

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.

  Funding Agency

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

  Institution

Name
University of North Carolina Chapel Hill
Department
Type
DUNS #
608195277
City
Chapel Hill
State
NC
Country
United States
Zip Code
27599

  Publications

Muhlebach, Marianne S; Zorn, Bryan T; Esther, Charles R et al. (2018) Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool children. PLoS Pathog 14:e1006798
Shobair, Mahmoud; Popov, Konstantin I; Dang, Yan L et al. (2018) Mapping allosteric linkage to channel gating by extracellular domains in the human epithelial sodium channel. J Biol Chem 293:3675-3684
Kota, Pradeep; Gentzsch, Martina; Dang, Yan L et al. (2018) The N terminus of ?-ENaC mediates ENaC cleavage and activation by furin. J Gen Physiol 150:1179-1187
Livraghi-Butrico, Alessandra; Wilkinson, Kristen J; Volmer, Allison S et al. (2018) Lung disease phenotypes caused by overexpression of combinations of ?-, ?-, and ?-subunits of the epithelial sodium channel in mouse airways. Am J Physiol Lung Cell Mol Physiol 314:L318-L331
Chen, Gang; Volmer, Allison S; Wilkinson, Kristen J et al. (2018) Role of Spdef in the Regulation of Muc5b Expression in the Airways of Naive and Mucoobstructed Mice. Am J Respir Cell Mol Biol 59:383-396
Abdullah, Lubna H; Coakley, Raymond; Webster, Megan J et al. (2018) Mucin Production and Hydration Responses to Mucopurulent Materials in Normal versus Cystic Fibrosis Airway Epithelia. Am J Respir Crit Care Med 197:481-491
Yu, Dongfang; Saini, Yogesh; Chen, Gang et al. (2018) Loss of ? Epithelial Sodium Channel Function in Meibomian Glands Produces Pseudohypoaldosteronism 1-Like Ocular Disease in Mice. Am J Pathol 188:95-110
Rowson-Hodel, A R; Wald, J H; Hatakeyama, J et al. (2018) Membrane Mucin Muc4 promotes blood cell association with tumor cells and mediates efficient metastasis in a mouse model of breast cancer. Oncogene 37:197-207
Terryah, Shawn T; Fellner, Robert C; Ahmad, Saira et al. (2018) Evaluation of a SPLUNC1-derived peptide for the treatment of cystic fibrosis lung disease. Am J Physiol Lung Cell Mol Physiol 314:L192-L205
Livraghi-Butrico, A; Grubb, B R; Wilkinson, K J et al. (2017) Contribution of mucus concentration and secreted mucins Muc5ac and Muc5b to the pathogenesis of muco-obstructive lung disease. Mucosal Immunol 10:829

Showing the most recent 10 out of 56 publications