A proportion of proteinase 3 (PR3)-specific anti-neutrophil cytoplasmic antibodies (ANCA) only react with PR3 after cleavage of its N-terminal activation dipeptide.

@article{Sun1998APO,
  title={A proportion of proteinase 3 (PR3)-specific anti-neutrophil cytoplasmic antibodies (ANCA) only react with PR3 after cleavage of its N-terminal activation dipeptide.},
  author={Jingwu Sun and David Fass and Margaret A Viss and Amber M. Hummel and Hsin-yi Y. Tang and HenryA. Homburger and Ulrich Specks},
  journal={Clinical and experimental immunology},
  year={1998},
  volume={114 2},
  pages={
          320-6
        }
}
ANCA directed against PR3 are highly specific for Wegener's granulomatosis and microscopic polyangiitis, and have been implicated in the pathogenesis of small vessel vasculitis. Most PR3-ANCA are directed against conformational epitopes on PR3. This study was designed to determine whether the cleavage of the N-terminal activation dipeptide of PR3 is required for the binding of PR3-ANCA. Recombinant PR3 (rPR3) variants were expressed in the epithelial cell line, 293. As confirmed by… CONTINUE READING
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