Alpha 1-antitrypsin genetic polymorphism in ANCA-positive systemic vasculitis.

  title={Alpha 1-antitrypsin genetic polymorphism in ANCA-positive systemic vasculitis.},
  author={Vincent Louis-Marie Esnault and Alessandra Testa and M. Audrain and Christophe Rog{\'e} and Mohamed Amine Hamidou and Jacques Henri Barrier and Richard Sesbo{\"u}{\'e} and J. Pinto – Martin and Philippe Lesavre},
  journal={Kidney international},
  volume={43 6},
Alpha 1-antitrypsin (alpha 1-AT) is the major inhibitor of proteinase 3 (PR3), the main target antigen of antineutrophil cytoplasm antibodies (ANCA) in Wegener's granulomatosis. alpha 1-AT is encoded by a polymorphic gene, with over 75 alleles, defining severely, medium and non-deficient protease inhibitor (PI) phenotypes. We describe the association of severely and medium deficient PI phenotypes with anti-PR3 positive systemic vasculitis, and postulate a pathogenetic role for alpha 1-AT… CONTINUE READING


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