Bimodal distribution of proteinase 3 (PR3) surface expression reflects a constitutive heterogeneity in the polymorphonuclear neutrophil pool

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Constitutive membrane expression of proteinase 3 (PR3) and neutrophil activation by anti-PR3 antibodies.

Antineutrophil cytoplasm autoantibodies with specificity for proteinase 3 (PR3) are thought to play a major role in the pathogenesis of Wegener's granulomatosis (WG), presumably by their potential to activate neutrophils. In patients with WG, high expression of PR3 on the surface of nonprimed neutrophils is associated with an increased incidence and rate of relapse. In this study, we analyzed t...

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A case of proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA) positive/IgG4-related lung disease

IgG4-related lung disease (IgG4-RLD) is a rare and chronic progressive autoimmune disease. We report a case of IgG4-related inflammatory pseudo-tumor of the lung that was seropositive for proteinase 3 anti-neutrophil cytoplasmic antibody (PR3-ANCA). A 61-year-old male had a mass lesion in the right lower lung field in chest X-ray. Transbronchial lung biopsy resulted in a pathological diagnosis ...

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Two Cases of Proteinase 3-Anti-Neutrophil Cytoplasmic Antibody (PR3-ANCA)-related Nephritis in Infectious Endocarditis

We herein report two cases of proteinase 3-anti-neutrophil cytoplasmic antibody (PR3-ANCA)-related nephritis in infectious endocarditis. In both cases, the patients were middle-aged men with proteinuria and hematuria, hypoalbuminemia, decreased kidney function, anemia, elevated C-reactive protein (CRP) levels, and PR3-ANCA positivity. Each had bacteremia, due to Enterococcus faecium in one and ...

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Membrane proteinase 3 expression on resting neutrophils as a pathogenic factor in PR3-ANCA-associated vasculitis.

Antineutrophil cytoplasm autoantibody (ANCA)-associated small vessel vasculitides are systemic diseases characterized by chronic inflammation of blood vessels. These vasculitides are associated with the presence of ANCA which are, in most cases, directed towards proteinase 3 (PR3) or myeloperoxidase (MPO). In vitro and in vivo data have suggested a pathophysiological role in the ANCA-associated...

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Effects of proteinase inhibitors on polymorphonuclear neutrophil polarization.

Polarization of polymorphonuclear neutrophils (PMNs) can be elicited by the chemotactic peptide N-formyl-methionyl-leucyl-phenylalanine (fMLP) and the microtubule-disrupting compound colchicine. Here we report on whether natural and synthetic proteinase inhibitors alter the polarizing response to these two agents. The alpha 1-proteinase inhibitor, N-tosyl-L-phenylalanine chloromethyl ketone, an...

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ژورنال

عنوان ژورنال: FEBS Letters

سال: 1995

ISSN: 0014-5793

DOI: 10.1016/0014-5793(95)01073-n