PM/Scl100
| Cat. No. | Available in | ![]() |
| 16000 | 0.1 mg | |
| 16001 | 1.0 mg | |
Diseases:
Scleroderma
Polymyositis and Dermatomyositis
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 is available from DIARECT by special order) 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.


