Catalogue number
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
EXOSC10 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 EXOSC10 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 EXOSC10 antibodies, making this specificity an important marker. The presence of EXOSC10 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
EXOSC10 Human Recombinant produced in SF9 is a glycosylated, polypeptide chain having a molecular mass of 102 kDa.
EXOSC10 is expressed with a -6xHis tag and purified by proprietary chromatographic techniques.
Source
Sf9 insect cells.
Physical Appearance
Sterile Filtered clear solution.
Formulation
EXOSC10 is 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.
Stability
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.
Safety Data Sheet
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.
