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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昆虫Sf9细胞中重组表达
溶解建议
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.
保存建议
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.
