Immunogen sequence: IAEELPKEP QGIIACCNPV PPLVRQQINE MHLLIQQARE MPLLKSEVAA GVKKSGPLPS AERLENVLFG PHDCSHAPPD GYPIIPTSGS VPVQKQASLF PDEKEDNLLG TTCLIATAVI TLFNEPSAED SKKGPLTVAQ KKAQNIMESF ENPFRMFLPS LGHRAPVSQA AKFDPSTKIY EISNRWKLAQ VQVQKDSKEA VKKKAAEQTA AREQAKEACK AAAEQAISVR QQVVLENAAK KRERATSDPR TTEQKQEKKR LKISKKPKDP EPPEKEFTPY DYSQSDFKAF AGNSKSKVSS QFDPNKQTPS GKKCIAAKKI KQSVGNKSMS FPTGKSDRGF RYNWPQR (540-885 aa encoded by BC073788 )
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; Autoantigen PM/Scl 2 homolog; Exosome component 10; P100 polymyositis-scleroderma overlap syndrome-associated autoantigen; PM/Scl-100; Polymyositis/scleroderma autoantigen 100 kDa; Polymyositis/scleroderma autoantigen 2; Polymyositis/scleroderma autoantigen 2 homolog
Gene Aliases: EXOSC10; p2; p3; p4; PM-Scl; PM/Scl-100; PMSCL; PMSCL2; RRP6; Rrp6p