Recombinant Human PM/Scl 100

PM/Scl 100 Human
PM/Scl 100 Human Recombinant
Catalog No. PRO-444

Synonyms:
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, RRP6, Rrp6p, PM-Scl.

Introduction:
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.

Description:
PM/Scl 100 Human Recombinant produced in SF9 is a glycosylated, polypeptide chain having a molecular mass of 120 kDa.
PM/Scl 100 is expressed with a -6xHis tag and purified by proprietary chromatographic techniques.

Source:
Sf9 insect cells.

Physical Appearance:
Sterile Filtered clear solution.

Formulation:
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.

Immunological functions:
1. Binds IgG-type human auto-antibodies.
2. Standard ELISA test (checker-board analysis of positive/negative sera panels).

Applications:
Western-Blot with monoclonal anti-hexa-His-tag antibody & polymyositis patient sera.

Coating concentration:
0.4-0.8 µg/ml (depending on the type of ELISA plate and coating buffer).
Suitable for biotinylation and iodination.

Storage Conditions:
Store at 4°C if entire vial will be used within 2-4 weeks.
Store, frozen at -20°C for longer periods of time.
Avoid multiple freeze-thaw cycles.

Purity:
Greater than 70% as determined by SDS-PAGE.

Usage:
Prospec's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.