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  • Title: Proteinase 3: substrate specificity and possible pathogenetic effect of Wegener's granulomatosis autoantibodies (c-ANCA) by dysregulation of the enzyme.
    Author: Dolman KM, van de Wiel BA, Kam CM, Kerrigan JE, Hack CE, von dem Borne AE, Powers JC, Goldschmeding R.
    Journal: Adv Exp Med Biol; 1993; 336():55-60. PubMed ID: 8296674.
    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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