FIGURE: 1 / 4
Recommended positive controls: 293T, EXOSC10-transfected 293T.
Predicted reactivity: Chimpanzee (99%).
Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.
About 50% of patients with polymyositis/scleroderma (PM-Scl) overlap syndrome are reported to have autoantibodies to a nuclear/nucleolar 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 patients. 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 ofthe RNA exosome complex which has 3'->5' exoribonuclease activity, EXOSC10 participates in a multitude of cellular RNA processing and degradation events.
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Protein Aliases: autoantigen PM-SCL; Autoantigen PM/Scl 2; Exosome component 10; P100 polymyositis-scleroderma overlap syndrome-associated autoantigen; PM/Scl-100; Polymyositis/scleroderma autoantigen 100 kDa; Polymyositis/scleroderma autoantigen 2
Gene Aliases: EXOSC10; p2; p3; p4; PM-Scl; PM/Scl-100; PMSCL; PMSCL2; RRP6; Rrp6p
UniProt ID: (Human) Q01780
Entrez Gene ID: (Human) 5394