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The human exosome: an autoantigenic complex of exoribonucleases in myositis and scleroderma

Rick Brouwer, Ger JM Pruijn and Walther J van Venrooij*

Author Affiliations

Department of Biochemistry, University of Nijmegen, Nijmegen, The Netherlands

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Arthritis Res 2001, 3:102-106  doi:10.1186/ar147

Published: 20 December 2000


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 3' → 5' exoribonucleases. To date, 10 human exosome components have been identified, although only some of these were studied in more detail. In this review, we discuss some recent advances in the characterization of the PM/Scl complex.

autoantibodies; autoantigens; exoribonucleases; exosome; PM/Scl complex