PM/Scl100

Cat. No.	Available in
16000	0.1 mg
16001	1 mg

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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.

PM/Scl 100 from DIARECT is expressed in the baculovirus expression system.