PM/Scl 100 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 PM/Scl 100 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 PM/Scl 100 antibodies, making this specificity an important marker. The presence of PM/Scl 100 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.
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來源宿主
昆蟲Sf9細胞中重組表達
溶解建議
Sterile Filtered clear solution. PM/Scl 100is supplied at a concentration of 0.8mg/ml in 20mM HEPES, pH-7.5, 100mM NaCl, 0.01mM EDTA, 0.02% SDS.
保存建議
Store at 4℃ if entire vial will be used within 2-4 weeks.
Store, frozen at -20℃ for longer periods of time.
Avoid multiple freeze-thaw cycles.