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Human Telomerase RNA Processing and Quality Control.
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About 50% of patients with polymyositis/scleroderma (PM-Scl) overlap syndrome are reported to have autoantibodies to a neuclear
ucleolar particle termed PM-Scl. Exosome component 10 (EXOSC10), also named autoantigen PM/Scl 2, is the 100 kDa antigen component of PM-Scl and is recognized by most sera of PM-Scl paitents. EXOSC10 is strongly enriched in the nucleolus and a small amount has been found in cytoplasm supporting the existence of a nucleolar RNA exosome complex form. As a putative catalytic component of the RNA exosome complex which has 3'->5' exoribonuclease activity, EXOSC10 participates in a multitude of cellular RNA processing and degradation events.