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Anti -ADAMTS-13 (Von Willebrand Factor-Cleaving Protease (VWFCP))
Anti -ADAMTS-13 (Von Willebrand Factor-Cleaving Protease (VWFCP))
Pricing
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| Clone |
Host |
Grade |
Applications |
| Monoclonal |
Mouse |
Affinity Purified |
E B IF |
|
| ADAMTS-13 is a zinc-containing metalloprotease belonging to the ADAMTS family characterized by a protease domain, an adjacent disintegrin-like domain, one or more thrombospondin type 1 repeats, a cystein-rich domain and a typical spacer region. ADAMTS-13 is composed of a series of domains (amino to carboxy terminal): metalloprotease, disintegrin-like, central thrombospondin-1 (TSP-1), cysteine-rich, spacer, seven additional TSP-1 domains and two unique CUB domains. ADAMTS-13 has no hydrophobic transmembrane domain, and hence it is not anchored in the cell membrane. The apparent molecular weight is 170 or 190kD on non-reducing or reducing SDS-PAGE, respectively. ADAMTS-13 has an important function in haemostasis, where it catalyzes the cleavage of the peptide bond between tyrosine-1605 and methionine-1606 in the A2 domain of von Willebrand Factor (VWF), resulting in 2 electrophoretic reduced fragments of 176 and 140kD, respectively. This process renders large multimers less adhesive and hence less reactive in the setting of thrombus formation. ADAMTS-13 is therefore said to be a natural anti-thrombotic agent. | | | Severe ADAMTS-13 deficiency is associated with systemic microvascular thrombosis in familial or acquired thrombotic thrombocytopenic purpura (TTP). The accumulation of non-cleaved large VWF multimers causes spontaneous systemic platelet aggregation blocking oxygen supply to vital organs. This life-threatening disorder can lead to ischemic disease with (multiple) organ failure. | | | Catalog # | A0859-61Z3 | | Applications | Suitable for use in Immunofluorescence, ELISA and Western Blot. Other applications have not been tested. | | Recommended Dilutions | | | ELISA: 1:50 | | Western Blot: 1:50 under non-reducing conditions. | | Immunofluorescence: 1:50 | | Optimal dilutions to be determined by the researcher. | | Storage and Stability | May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. | | CAS Number | n/a | | Clone Type | Monoclonal | | Isotype | IgG1 | | Clone No | 20A5 | | Host | Mouse | | Concentration | ~0.1mg/ml | | Form | Supplied as as liquid in PBS, 0.1% BSA, 0.02% sodium azide. | | Purity | Purified by Protein G affinity chromatography. | | Immunogen | Recombinant corresponding to full length ADAMTS-13. | | Specificity | Recognizes ADAMTS-13. Reacts with the central to C-terminal TSP-1 repeats 2-5 of ADAMTS-13 (aa686-894). Species Crossreactivity: Human | | | Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological. |
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