Proteinase 3: substrate specificity and possible pathogenetic effect of Wegener's granulomatosis autoantibodies (c-ANCA) by dysregulation of the enzyme.
Proteinase 3: substrate specificity and possible pathogenetic effect of Wegener's granulomatosis autoantibodies (c-ANCA) by dysregulation of the enzyme.
复制标题
蛋白酶 3：酶失调导致韦格纳肉芽肿自身抗体 (c-ANCA) 的底物特异性和可能的致病作用。
DOI：
10.1007/978-1-4757-9182-2_7
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发表时间：
1993
影响因子：
--
通讯作者：
Goldschmeding,R
中科院分区：
文献类型：
--
作者：
Dolman,KM;vandeWiel,BA;Kam,CM;Kerrigan,JE;Hack,CE;vondemBorne,AE;Powers,JC;Goldschmeding,R
Reactivity of proteinase 3 (PR3) was tested against various amino acid and thioester substrates. The best substrate is Boc-Ala-Ala-Nva-SBzl with akcat/Kmvalue of 1.0 × 106M−1.s−1. We also studied the effect of C-ANCA on PR3 proteolytic activity towards elastin and inactivation by alphas-antitrypsin (α1AT). 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 α1AT.
