Dr. Specks is the principal investigator (PI) of laboratory-based research studies designed to characterize the role of the neutrophil serine proteases, proteinase 3 (PR3) and elastase in the pathogenesis of Wegener's granulomatosis, vasculitis and lung fibrosis. Specific aims of these studies are to identify epitopes on the PR3 molecule which are preferentially recognized by PR3-ANCA (autoantibodies from patients with Wegener's granulomatosis), to characterize the effect of such epitope-specific PR3-ANCA on biological functions of PR3, and to determine whether the functional effects of PR3-ANCA have a bearing on specific clinical disease manifestations. Furthermore, the laboratory investigates structure-function relationships of PR3 and elastase and their significance in the pathogenesis of lung fibrosis. These studies have been supported by grants from the American Heart Association, the NIH and the Robert N. Brewer Foundation.