Identity of Wegener's autoantigen (p29) with proteinase 3 and myeloblastin.
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چکیده
منابع مشابه
Wegener's granulomatosis autoantigen is a novel neutrophil serine proteinase.
Circulating IgG autoantibodies that produce cytoplasmic immunofluorescence staining of ethanol-fixed normal neutrophils have recently been found in a large percentage of patients with active Wegener's granulomatosis. Such autoantibodies are rarely found in other diseases and are therefore virtually diagnostic of Wegener's granulomatosis. The nature of the neutrophil antigen defined by these aut...
متن کاملWegener's granulomatosis--autoimmunity to neutrophil proteinase 3.
Wegener's granulomatosis (WG) is a small-vessel vasculitis associated with various clinical manifestations, among which the most common are respiratory tract disease and glomerulonephritis leading to renal failure. The pathogenesis of vascular injury in WG is ascribed to antineutrophil cytoplasmic antibodies (ANCA) directed mainly against proteinase 3 (PR3), an enzyme from neutrophil granules. ...
متن کاملPresence of proteinase 3 in secretory vesicles: evidence of a novel, highly mobilizable intracellular pool distinct from azurophil granules.
Proteinase 3 (PR3), which is also called myeloblastin, the target autoantigen for antineutrophil cytoplasmic antibodies (ANCA) in Wegener's granulomatosis, is a serine proteinase stored in azurophil granules of human neutrophils. We have previously shown that, in contrast to elastase or myeloperoxidase, PR3 is also expressed at the plasma membrane of a subset of unactivated neutrophils and that...
متن کاملProteinase 3, Wegener's autoantigen: from gene to antigen.
Proteinase 3 (PR3) is one of four serine protease homologues in the azurophilic granules of neutrophils and granules of monocytes. It is of importance that anti-neutrophil cytoplasmic antibodies (ANCA) in patients with Wegener's granulomatosis (WG) are mainly directed against PR3 only. Furthermore, PR3 is overexpressed in a variety of acute and chronic myeloid leukemia cells. Cytotoxic T lympho...
متن کاملpH-dependent hysteretic behaviour of human myeloblastin (leucocyte proteinase 3).
Human myeloblastin (leucocyte proteinase 3) showed a very slow approach to the steady-state velocity when the pH was rapidly increased from 3.2 to 7.0. The kinetic mechanism of this hysteretic process was interpreted as a slow conformational change of myeloblastin from an inactive form at acidic pH to the active form at neutral pH. The transition between the two enzyme forms could occur spontan...
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ورودعنوان ژورنال:
- Blood
دوره 76 10 شماره
صفحات -
تاریخ انتشار 1990