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| Uniprot ID : |
Q01780 |
| Alternative Name : |
Exosome component 10, Polymyositis/scleroderma autoantigen 2, Autoantigen PM/Scl 2, Polymyositis/scleroderma autoantigen 100 kDa, PM/Scl-100, P100 polymyositis-scleroderma overlap syndrome-associated autoantigen, EXOSC10, PMSCL, PMSCL2, p2, p3, p4, R |
| Amount : |
20 µg |
Source : Sf9 insect cells. EXOSC10 Human Recombinant produced in SF9 is a glycosylated, polypeptide chain having a molecular mass of 120 kDa. EXOSC10 is expressed with a -6xHis tag and purified by proprietary chromatographic techniques. EXOSC10 is a nucleolar protein and represents one crucial component of the so-called 'PM/Scl complex' or 'nuclear exosome', which is an RNA-processing complex consisting of a number of exoribonucleases (not to be confused with certain multivesicular bodies which are shed by cultured cells into the culture fluid and are also called exosomes).Autoantibodies against EXOSC10 occur in patients suffering from polymyositis, scleroderma or polymyositis/scleroderma overlap syndromes. While antibodies to other components of the exosome complex (PM/Scl 75, several Rrp proteins) can be found in some sera, practically all patients with autoantibodies against the PM/Scl complex are positive for EXOSC10 antibodies, making this specificity an important marker. The presence of EXOSC10 autoantibodies is a comparatively 'good' prognostic sign in polymyositis/scleroderma overlap syndrome unlike the poor prognosis seen when other myositis and systemic sclerosis specific autoantibodies are present.
