Literature DB >> 8296674

Proteinase 3: substrate specificity and possible pathogenetic effect of Wegener's granulomatosis autoantibodies (c-ANCA) by dysregulation of the enzyme.

K M Dolman1, B A van de Wiel, C M Kam, J E Kerrigan, C E Hack, A E von dem Borne, J C Powers, R Goldschmeding.   

Abstract

Reactivity of proteinase 3 (PR3) was tested against various amino acid and thioester substrates. The best substrate is Boc-Ala-Ala-Nva-SBzl with a kcat/Km value of 1.0 x 10(6) M-1.s-1. We also studied the effect of C-ANCA on PR3 proteolytic activity towards elastin and inactivation by alpha 1-antitrypsin (alpha 1AT). C-ANCA IgG from 8 patients with active Wegener's granulomatosis were tested and found to inhibit elastin degradation by PR3 and to prevent the inactivation of PR3 by alpha 1AT.

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Year:  1993        PMID: 8296674     DOI: 10.1007/978-1-4757-9182-2_7

Source DB:  PubMed          Journal:  Adv Exp Med Biol        ISSN: 0065-2598            Impact factor:   2.622


  3 in total

Review 1.  Pathogenesis and treatment of ANCA-associated systemic vasculitis.

Authors:  A D Salama
Journal:  J R Soc Med       Date:  1999-09       Impact factor: 5.344

Review 2.  Natural catalytic antibodies.

Authors:  S Paul
Journal:  Mol Biotechnol       Date:  1996-06       Impact factor: 2.695

3.  Anti-neutrophil cytoplasmic antibodies (ANCA) from patients with systemic vasculitis recognize restricted epitopes of proteinase 3 involving the catalytic site.

Authors:  M E Griffith; A Coulthart; S Pemberton; A J George; C D Pusey
Journal:  Clin Exp Immunol       Date:  2001-01       Impact factor: 4.330

  3 in total

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