the human exosome an autoantigenic complex of exoribonucleases in myositis and scleroderma人类的外来体的autoantigenic复杂exoribonucleases肌炎和硬皮病.pdf

Available online /content/3/2/102 Review The human exosome: an autoantigenic complex of exoribonucleases in myositis and scleroderma Rick Brouwer, Ger J M Pruijn and Walther J van Venrooij Department of Biochemistry, University of Nijmegen, Nijmegen, The Netherlands Correspondence: Dr W J van Venrooij, Department of Biochemistry, University of Nijmegen, PO Box 9101, NL-6500 HB, Nijmegen, The Netherlands. Tel: +31 24 3613656; fax: +31 24 3540525; e-mail: w.vanvenrooij@bioch.kun.nl Received: 1 November 2000 Arthritis Res 2001, 3:102–106 Revisions requested: 17 November 2000 This article may contain supplementary data which can only be found Revisions received: 20 November 2000 online at /content/3/2/102 Accepted: 22 November 2000 © BioMed Central Ltd on behalf of the copyright holder Published: 20 December 2000 (Print ISSN 1465-9905; Online ISSN 1465-9913) Abstract The anti-PM/Scl autoantibodies are known to characterize a subset of autoimmune patients with myositis, scleroderma (Scl), and the PM/Scl overlap syndrome. The major autoantigens that are recognized by anti-PM/Scl autoantibodies are designated PM/Scl-100 and PM/Scl-75. These autoantigens have been reported to associate into a large complex consisting of 11 to 16 proteins and to play a role in ribosome synthesis. Recently, it was discovered that the PM/Scl complex is the human counterpart of the yeast (Saccharomyces cerevisiae) exosome, which is an RNA-processing complex consisting of 11 35 exoribonucleases. To date, 10 human exosome