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Updated: Aug 9, 2026

Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
The human exosome: an autoantigenic complex of exoribonucleases in myositis and scleroderma
R Brouwer1, G J Pruijn, W J van Venrooij
1Department of Biochemistry, University of Nijmegen, NL-6500 HB Nijmegen, The Netherlands.
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 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.
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