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Membrane proteinase 3 and Wegener's granulomatosis

Item Type:Article
Title:Membrane proteinase 3 and Wegener's granulomatosis
Creators Name:von Vietinghoff, S. and Schreiber, A. and Otto, B. and Choi, M. and Goebel, U. and Kettritz, R.
Abstract:Proteinase 3 (PR3) is found in neutrophil and monocyte lysosomal granules. Anti-neutrophil cytoplasmatic antibodies (ANCA) with specificity for PR3 are characteristic for patients with Wegener's granulomatosis. The interaction of ANCA with neutrophilic ANCA antigens is necessary for the development of ANCA-associated diseases. ANCA bind to membrane-expressed PR3 and induce full-blown activation in primed neutrophils. We discuss two different aspects of membrane PR3 (mPR3). The first aspect is the amount of PR3 and mechanisms controlling this issue. The second aspect is the presence of two neutrophil subsets that differ in the mPR3 expression phenotype.
Keywords:Membrane Proteinase 3, PR3 ANCA, Wegeners Granulomatosis
Source:Clinical Nephrology
Page Range:453-459
Date:1 January 2005
PubMed:View item in PubMed

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