Proteinase 3: Substrate Specificity and Possible Pathogenetic Effect of Wegener’s Granulomatosis Autoantibodies (C-ANCA) by Dysregulation of the Enzyme

  • Koert M. Dolman
  • Bart A. van de Wiel
  • Chih-Min Kam
  • John E. Kerrigan
  • C. Erik Hack
  • Albert E. G. Kr. von dem Borne
  • James C. Powers
  • Roel Goldschmeding
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 336)

Summary

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 k cat/ K m value of 1.0 × 106 M−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.

Keywords

Neutrophil Elastase Positive Seron Primed Neutrophil Neutrophil Serine Proteinase Peptide Thioester 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media New York 1993

Authors and Affiliations

  • Koert M. Dolman
    • 1
  • Bart A. van de Wiel
    • 1
  • Chih-Min Kam
    • 4
  • John E. Kerrigan
    • 4
  • C. Erik Hack
    • 1
  • Albert E. G. Kr. von dem Borne
    • 1
    • 2
  • James C. Powers
    • 4
  • Roel Goldschmeding
    • 1
    • 3
  1. 1.Central Laboratory of the Netherlands Red Cross Blood Transfusion Service Laboratory for Experimental and Clinical ImmunologyUniversity of AmsterdamAmsterdamThe Netherlands
  2. 2.Departments of Hematology Academic Medical CentreUniversity of AmsterdamAmsterdamThe Netherlands
  3. 3.Departments of Pathology Academic Medical CentreUniversity of AmsterdamAmsterdamThe Netherlands
  4. 4.School of Chemistry and BiochemistryGeorgia Institute of TechnologyAtlantaUSA

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