Summary
Classic anti-neutrophil cytoplasmic antibodies (c-ANCA) specific for constituents of neutrophil primary granules and monocyte lysosomes have been implicated in the pathogenesis of Wegener’s Granulomatosis (WG). The revised amino-terminal sequence of Proteinase 3 (PR-3) as ANCA antigen, suggested that PR-3 is identical to myeloblastin (MBN). As it has been proposed that autoantibodies recognize a conformational epitope on c-ANCA, prokaryotic expressed protein might not be recognized by the patients sera. Therefore we set up an in vitro translation in an eukaryotic cell-free system using an internal ATG (amino acid 15 of the mature protein).
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References
Van der Woude, F. J., Lobatto, S., Permin, H., van der Giessen, M., Rasmussen, N., Wiik, A., van Es, L. A., van der Hem, G. K., and The, T. H., 1985, Autoantibodies against neutrophils and monocytes: Tool for diagnosis and marker of disease activity in Wegener’s granulomatosis. Lancet 1: 425-429.
Bleil, L., Manger, B., Winkler, T. H., Herrman, M., Burmester, G. R., Krapf, F. E., and Kalden, J. R., 1991, The role of antineutrophil cytoplasm antibodies, anticardiolipin antibodies, von Willebrand factor antigen, and fibronectin for the diagnosis of systemic vasculitis, J. Rheumatol. 18: 1199 - 1206.
Kao, R. C., Wehner, N. G., Skubitz, K. M., Gray, B. H., and Hoidal, J. R., 1988, A distinct human polymorphonuclear leukocyte proteinase that produces emphysema in hamsters, J. Clin. Invest. 82: 1963-1973.
Lüdemann, J., Utecht, B., and Gross, W. L., 1990, Anti-neutrophil cytoplasm antibodies in Wegener’s granulomatosis recognize an elastinolytic enzyme, J. Exp. Med. 171: 357-362.
Jenne, D. E., Tschopp, J., Lüdemann, J., Utecht, B., and Gross, W. L., 1990, Wegener’s autoantigen decoded, Nature 346: 520 (correspondence).
Niles, J. L., McCluskey, R. T., Ahmad, M. F., and Amaout, M A., 1989, Wegener’s granulomatosis autoantigen is a novel neutrophil serine proteinase, Blood 74: 1888 - 1893.
Gupta, S. K., Niles, J. L., McCluskey, R. T., and Arnaout, M. A., 1990, Identity of Wegener’s autoantigen (P29) with proteinase 3 and myeloblastin, Blood 76: 2162 (correspondence).
Bories, D., Raynal, M.-C., Solomon, D. H., Darzynkiewicz, Z., and Cayre, Y. E., 1989, Down-regulation of a serine protease, myeloblastin, causes growth arrest and differentiation of promyelocytic leukemia cells, Cell 59: 959 - 968.
Kozak, M., 1986, Point mutations define a sequence flanking the AUG initiator codon that modulates translation by eukaryotic ribosomes, Cell 44: 283 - 292.
Labbaye, C., Musette, P, and Cayre, Y. E., 1991, Wegener autoantigen and myeloblastin are encoded by a single mRNA, Proc. Natl. Acad. Sci. USA 88: 9253-9256.
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Bleil, L., Winkler, T.H., Fehr, H.K., Bautz, E.K.F., Kalden, J.R. (1993). A New Approach to the Molecular Characterization of the C-ANCA Antigen in Wegener’s Granulomatosis. In: Gross, W.L. (eds) ANCA-Associated Vasculitides. Advances in Experimental Medicine and Biology, vol 336. Springer, Boston, MA. https://doi.org/10.1007/978-1-4757-9182-2_6
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DOI: https://doi.org/10.1007/978-1-4757-9182-2_6
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