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

被引:55
作者
Brouwer, R [1 ]
Pruijn, GJM [1 ]
van Venrooij, WJ [1 ]
机构
[1] Univ Nijmegen, Dept Biochem, NL-6500 HB Nijmegen, Netherlands
关键词
autoantibodies; autoantigens; exoribonucleases; exosome; PM/Scl complex;
D O I
10.1186/ar147
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:102 / 106
页数:5
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