scholarly journals Proteinase 3 associated with Wegener’s Granulomatosis

2021 ◽  
Author(s):  
Wolfgang H. Goldmann
Keyword(s):  
Wegener’S Granulomatosis ◽  
Wegener's Granulomatosis ◽  
Proteinase 3

A substrate‐based approach to convert SerpinB1 into a specific inhibitor of proteinase 3, the Wegener's granulomatosis autoantigen

2011 ◽  
Vol 25 (9) ◽  
pp. 3019-3031 ◽  
Author(s):  
Gwenhael Jégot ◽  
Chrystelle Derache ◽  
Sandrine Castella ◽  
Hichem Lahouassa ◽  
Elodie Pitois ◽  
...  
Keyword(s):  
Wegener’S Granulomatosis ◽  
Specific Inhibitor ◽  
Wegener's Granulomatosis ◽  
Proteinase 3

Wegener's Granulomatosis and Antineutrophil Cytoplasmic Autoantibodies

1993 ◽  
Vol 102 (11) ◽  
pp. 906-908 ◽  
Author(s):  
John G. Batsakis ◽  
Adel K. El-Naggar
Keyword(s):  
Laboratory Test ◽  
Wegener’S Granulomatosis ◽  
Wegener's Granulomatosis ◽  
Proteinase 3 ◽  
Inflammatory Disorders ◽  
Serologic Tests ◽  
Criteria For Diagnosis ◽  
Antineutrophil Cytoplasmic Autoantibodies

Serologic tests for antineutrophil cytoplasmic autoantibodies can serve as markers for a number of necrotizing vasculitides and other inflammatory disorders. In the case of Wegener's granulomatosis, an immunofluorescent cytoplasmic pattern and immunoassay for proteinase 3 are quite specific for the disease and are capable of serving as additional criteria for diagnosis. As with any laboratory test, however, results are to be interpreted in the whole clinical and pathologic framework of the disease.

scholarly journals Wegener's Granulomatosis: Anti–proteinase 3 Antibodies Are Potent Inductors of Human Endothelial Cell Signaling and Leakage Response

1998 ◽  
Vol 187 (4) ◽  
pp. 497-503 ◽  
Author(s):  
Ulf Sibelius ◽  
Katja Hattar ◽  
Angelika Schenkel ◽  
Thomas Noll ◽  
Elena Csernok ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Wegener’S Granulomatosis ◽  
Inositol Phosphates ◽  
Vascular Endothelial Cells ◽  
Platelet Activating Factor ◽  
Barrier Properties ◽  
Wegener's Granulomatosis ◽  
Phosphoinositide Hydrolysis ◽  
Proteinase 3 ◽  
Dose Dependent

Anti–neutrophil cytoplasmic antibodies (ANCAs) targeting proteinase 3 (PR3) have a high specifity for Wegener's granulomatosis (WG), and their role in activating leukocytes is well appreciated. In this study, we investigated the influence of PR3-ANCA and murine monoclonal antibodies on human umbilical vascular endothelial cells (HUVECs). Priming of HUVECs with tumor necrosis factor α induced endothelial upregulation of PR3 message and surface expression of this antigen, as measured by Cyto-ELISA, with a maximum occurrence after 2 h. Primed cells responded to low concentrations of both antibodies (25 ng–2.5 μg/ml), but not to control immunoglobulins, with pronounced, dose-dependent phosphoinositide hydrolysis, as assessed by accumulation of inositol phosphates. The signaling response peaked after 20 min, in parallel with the appearance of marked prostacyclin and platelet-activating factor synthesis. The F(ab)2 fragment of ANCA was equally potent as ANCA itself. Disrupture of the endothelial F-actin content by botulinum C2 toxin to avoid antigen–antibody internalization did not affect the response. In addition to the metabolic events, anti-PR3 challenge, in the absence of plasma components, provoked delayed, dose-dependent increase in transendothelial protein leakage. We conclude that anti-PR3 antibodies are potent inductors of the preformed phosphoinositide hydrolysis–related signal tranduction pathway in human endothelial cells. Associated metabolic events and the loss of endothelial barrier properties suggest that anti-PR3–induced activation of endothelial cells may contribute to the pathogenetic sequelae of autoimmune vasculitis characterizing WG.

scholarly journals Mapping of Conformational Epitopes on Human Proteinase 3, the Autoantigen of Wegener’s Granulomatosis

2010 ◽  
Vol 185 (1) ◽  
pp. 387-399 ◽  
Author(s):  
Angelika Kuhl ◽  
Brice Korkmaz ◽  
Bert Utecht ◽  
Andrea Kniepert ◽  
Ulf Schönermarck ◽  
...  
Keyword(s):  
Wegener’S Granulomatosis ◽  
Wegener's Granulomatosis ◽  
Proteinase 3 ◽  
Conformational Epitopes
Sign in / Sign up

Export Citation Format

Share Document