蛋白酶3：韦格纳肉芽肿自身抗体（c-ANCA）通过酶的失调作用的底物特异性及可能的致病效应
Proteinase 3: substrate specificity and possible pathogenetic effect of Wegener's granulomatosis autoantibodies (c-ANCA) by dysregulation of the enzyme.
作者信息
Dolman K M, van de Wiel B A, Kam C M, Kerrigan J E, Hack C E, von dem Borne A E, Powers J C, Goldschmeding R
机构信息
Central Laboratory of The Netherlands, Red Cross Blood Transfusion Service, Amsterdam.
出版信息
Adv Exp Med Biol. 1993;336:55-60. doi: 10.1007/978-1-4757-9182-2_7.
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.
针对各种氨基酸和硫酯底物测试了蛋白酶3（PR3）的反应活性。最佳底物是Boc-Ala-Ala-Nva-SBzl，其催化常数与米氏常数的比值（kcat/Km）为1.0×10⁶ M⁻¹·s⁻¹。我们还研究了C-ANCA对PR3针对弹性蛋白的蛋白水解活性以及α1-抗胰蛋白酶（α1AT）使其失活的影响。对8例活动性韦格纳肉芽肿患者的C-ANCA IgG进行了测试，发现其可抑制PR3对弹性蛋白的降解，并防止α1AT使PR3失活。
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