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.
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.
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|Kropski, Jonathan A; Blackwell, Timothy S (2018) Endoplasmic reticulum stress in the pathogenesis of fibrotic disease. J Clin Invest 128:64-73|
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|Juge, Pierre-Antoine; Lee, Joyce S; Ebstein, Esther et al. (2018) MUC5B Promoter Variant and Rheumatoid Arthritis with Interstitial Lung Disease. N Engl J Med 379:2209-2219|
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