CORE B: The Clinical Ascertainment and Phenotyping Core will establish the clinical and phenotypic classifications which are pivotal for the Program. Core B will also manage all of the biologic resources for the Program. The goal of Core B is to recruit patients and members of families with familial interstitial pneumonia (FIP), to characterize all phenotypes, and obtain the specimens needed to conduct all studies ofthe Program. Core B personnel and facilities will perform the following key functions: 1) patient and family ascertainment, 2) recruitment, 3) phenotyping, 4) database maintenance, 5) specimen acquisition and banking, 6) specimen retrieval and distribution, 7) telomere length analyses. Core B study coordinators at all 3 sites (Vanderbilt, National Jewish/Univ Colorado, and Duke) enter and maintain a detailed database in Progeny Software (Wolfville, Nova Scotia), including pedigree information on each family and phenotype on each individual. Core B laboratory personnel will be responsible for specimen processing, cell culture, storage and management of specimen data. Core B will provide biomaterials (serum, lymphocytes, lymphoblastoids, lung tissue) and high throughput molecular biology services (telomere length analyses) to Program investigators.

Public Health Relevance

Interstitial lung diseases, including the idiopathic interstitial pneumonias, are a substantial cause of morbidity and mortality for which there are no effective treatments. In this program, we will study the genetics and underlying biological mechanisms that lead to progressive fibrosis in the lungs. Our integrated approach will lead to new concepts in disease pathogenesis and identification of novel treatment strategies.

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Research Program Projects (P01)
Project #
5P01HL092870-05
Application #
8598503
Study Section
Heart, Lung, and Blood Initial Review Group (HLBP)
Project Start
Project End
Budget Start
2014-01-01
Budget End
2014-12-31
Support Year
5
Fiscal Year
2014
Total Cost
$344,567
Indirect Cost
$97,781
Name
Vanderbilt University Medical Center
Department
Type
DUNS #
004413456
City
Nashville
State
TN
Country
United States
Zip Code
37212
Mathai, Susan K; Schwartz, David A; Warg, Laura A (2014) Genetic susceptibility and pulmonary fibrosis. Curr Opin Pulm Med 20:429-35
Molyneaux, Phillip L; Cox, Michael J; Willis-Owen, Saffron A G et al. (2014) The role of bacteria in the pathogenesis and progression of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 190:906-13
Shi, Chanjuan; Washington, M Kay; Chaturvedi, Rupesh et al. (2014) Fibrogenesis in pancreatic cancer is a dynamic process regulated by macrophage-stellate cell interaction. Lab Invest 94:409-21
Kropski, Jonathan A; Mitchell, Daphne B; Markin, Cheryl et al. (2014) A novel dyskerin (DKC1) mutation is associated with familial interstitial pneumonia. Chest 146:e1-7
Kropski, Jonathan A; Lawson, William E; Young, Lisa R et al. (2013) Genetic studies provide clues on the pathogenesis of idiopathic pulmonary fibrosis. Dis Model Mech 6:9-17
Atochina-Vasserman, Elena N; Biktasova, Asel; Abramova, Elena et al. (2013) Aquaporin 11 insufficiency modulates kidney susceptibility to oxidative stress. Am J Physiol Renal Physiol 304:F1295-307
Peljto, Anna L; Zhang, Yingze; Fingerlin, Tasha E et al. (2013) Association between the MUC5B promoter polymorphism and survival in patients with idiopathic pulmonary fibrosis. JAMA 309:2232-9
Hunninghake, Gary M; Hatabu, Hiroto; Okajima, Yuka et al. (2013) MUC5B promoter polymorphism and interstitial lung abnormalities. N Engl J Med 368:2192-200
Lawson, William E; Blackwell, Timothy S (2013) ýý-Catenin and CCNs in lung epithelial repair. Am J Physiol Lung Cell Mol Physiol 304:L579-81
Seibold, Max A; Smith, Russell W; Urbanek, Cydney et al. (2013) The idiopathic pulmonary fibrosis honeycomb cyst contains a mucocilary pseudostratified epithelium. PLoS One 8:e58658

Showing the most recent 10 out of 24 publications