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 #
5P01HL092870-04
Application #
8463841
Study Section
Heart, Lung, and Blood Initial Review Group (HLBP)
Project Start
Project End
Budget Start
2013-01-01
Budget End
2013-12-31
Support Year
4
Fiscal Year
2013
Total Cost
$364,996
Indirect Cost
$103,952
Name
Vanderbilt University Medical Center
Department
Type
DUNS #
004413456
City
Nashville
State
TN
Country
United States
Zip Code
37212
Sivley, R Michael; Sheehan, Jonathan H; Kropski, Jonathan A et al. (2018) Three-dimensional spatial analysis of missense variants in RTEL1 identifies pathogenic variants in patients with Familial Interstitial Pneumonia. BMC Bioinformatics 19:18
Burman, Ankita; Kropski, Jonathan A; Calvi, Carla L et al. (2018) Localized hypoxia links ER stress to lung fibrosis through induction of C/EBP homologous protein. JCI Insight 3:
Wilfong, Erin M; Lentz, Robert J; Guttentag, Adam et al. (2018) Interstitial Pneumonia With Autoimmune Features: An Emerging Challenge at the Intersection of Rheumatology and Pulmonology. Arthritis Rheumatol 70:1901-1913
Celada, Lindsay J; Kropski, Jonathan A; Herazo-Maya, Jose D et al. (2018) PD-1 up-regulation on CD4+ T cells promotes pulmonary fibrosis through STAT3-mediated IL-17A and TGF-?1 production. Sci Transl Med 10:
Hewlett, Justin C; Kropski, Jonathan A; Blackwell, Timothy S (2018) Idiopathic pulmonary fibrosis: Epithelial-mesenchymal interactions and emerging therapeutic targets. Matrix Biol 71-72:112-127
Kropski, Jonathan A; Blackwell, Timothy S (2018) Endoplasmic reticulum stress in the pathogenesis of fibrotic disease. J Clin Invest 128:64-73
Evans, Christopher M; Dickey, Burton F; Schwartz, David A (2018) E-Cigarettes: Mucus Measurements Make Marks. Am J Respir Crit Care Med 197:420-422
Lentz, Robert J; Taylor, Trevor M; Kropski, Jonathan A et al. (2018) Utility of Flexible Bronchoscopic Cryobiopsy for Diagnosis of Diffuse Parenchymal Lung Diseases. J Bronchology Interv Pulmonol 25:88-96
Brittain, Evan L; Thennapan, Thennapan; Maron, Bradley A et al. (2018) Update in Pulmonary Vascular Disease 2016 and 2017. Am J Respir Crit Care Med 198:13-23
Kook, Seunghyi; Qi, Aidong; Wang, Ping et al. (2018) Gene-edited MLE-15 Cells as a Model for the Hermansky-Pudlak Syndromes. Am J Respir Cell Mol Biol 58:566-574

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