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| Catalog # | Availability | Size | Quantity | Unit Price | Save For Later Wish List | |
|---|---|---|---|---|---|---|
| RCP0787-2 | 7 days | 2 µg | $120.00 |
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| RCP0787-10 | 7 days | 10 µg | $290.00 | |||
| RCP0787-100 | 7 days | 100 µg | $1,920.00 |
Product Overview | |
| Name | EXOSC1 Human |
|---|---|
| Description | |
| Exosome Component 1 Human Recombinant | |
| Accession (Primary) | Q9Y3B2 |
| 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. | |
| Source | |
| Sf9 insect cells. | |
| Physical Appearance | |
| Sterile Filtered clear solution. | |
| Formulation | |
| EXOSC10 is supplied in 20mM HEPES, pH-7.5, 100mM NaCl, 0.01mM EDTA, 0.02% SDS. | |
| 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. | |
| Purity | |
| Greater than 95% as determined by SDS-PAGE. | |
| Applications | |
| Western-Blot with monoclonal anti-hexa-His-tag antibody & polymyositis patient sera. | |
| Immunological functions | |
| 1. Binds IgG-type human auto-antibodies. 2. Standard ELISA test (checker-board analysis of positive/negative sera panels). | |
| Coating concentration | |
| 0.4-0.8 µg/ml (depending on the type of ELISA plate and coating buffer). Suitable for biotinylation and iodination. | |
| Precautions | |
| EXOSC1 Human is for research use only and not for use in diagnostic or therapeutic procedures. | |
Target Information: ( Q9Y3B2 ) | |
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ADMEbio Team
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