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

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.

• Authors: R Brouwer, G J Pruijn, W J van Venrooij
• Journal: Arthritis research (Arthritis Res) Vol. 3 Issue 2 Pg. 102-6 ( 2001) ISSN: 1465-9905 [Print] England
• PMID: 11178117 (Publication Type: Journal Article, Review)

Chemical References

• Autoantigens
• Multienzyme Complexes
• Exoribonucleases
• Exosome Multienzyme Ribonuclease Complex
• EXOSC10 protein, human

Topics

• Autoantigens (chemistry, immunology)
• Exoribonucleases (chemistry, immunology)
• Exosome Multienzyme Ribonuclease Complex
• Humans
• Multienzyme Complexes (chemistry, immunology)
• Polymyositis (enzymology, immunology)
• Scleroderma, Systemic (enzymology, immunology)