Characterization of proteinase-3 (PR-3), a neutrophil serine proteinase. Structural and functional properties

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Characterization of proteinase-3 (PR-3), a neutrophil serine proteinase. Structural and functional properties.

Proteinase 3 (PR-3) is a human polymorphonuclear leukocyte (PMNL) serine proteinase that degrades elastin in vitro and causes emphysema when administered by tracheal insufflation to hamsters (Kao, R. C., Wehner, N. G., Skubitz, K. M., Gray, B. H., and Hoidal, J. R. (1988) J. Clin. Invest. 82, 1963-1973). We have determined the primary structure of several PR-3 peptides and have analyzed catalyt...

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Wegener'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. ...

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Neutrophil elastase and proteinase-3 trigger G protein-biased signaling through proteinase-activated receptor-1 (PAR1).

Neutrophil proteinases released at sites of inflammation can affect tissue function by either activating or disarming signal transduction mediated by proteinase-activated receptors (PARs). Because PAR1 is expressed at sites where abundant neutrophil infiltration occurs, we hypothesized that neutrophil-derived enzymes might also regulate PAR1 signaling. We report here that both neutrophil elasta...

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Anti-neutrophil cytoplasm antibodies (ANCA) are useful diagnostic markers in a range of small vessel vasculitides. While non-specific ANCA have been reported in association with a variety of autoimmune, haematological and infectious conditions, the combination of a cytoplasmic staining pattern on indirect immunofluorescence (IIF) of human neutrophils, with antibodies specific for proteinase 3 (...

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ژورنال

عنوان ژورنال: Journal of Biological Chemistry

سال: 1991

ISSN: 0021-9258

DOI: 10.1016/s0021-9258(18)92854-1