The human exosome: an autoantigenic complex of exoribonucleases in myositis and scleroderma.
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.
Brouwer
R
R
Department of Biochemistry, University of Nijmegen, NL-6500 HB Nijmegen, The Netherlands.
Pruijn
G J
GJ
van Venrooij
W J
WJ
D001324:Autoantigens / Q000737:chemistry / Q000276:immunology*; D005095:Exoribonucleases / Q000737:chemistry / Q000276:immunology*; D063326:Exosome Multienzyme Ribonuclease Complex; D006801:Humans; D009097:Multienzyme Complexes / Q000737:chemistry / Q000276:immunology*; D017285:Polymyositis / Q000201:enzymology* / Q000276:immunology*; D012595:Scleroderma, Systemic / Q000201:enzymology* / Q000276:immunology*
D001324:Autoantigens; D009097:Multienzyme Complexes; D005095:Exoribonucleases; D063326:Exosome Multienzyme Ribonuclease Complex; C077044:EXOSC10 protein, human