A0859-61Z3
ADAMTS-13 (Von Willebrand Factor-Cleaving Protease, VWFCP)
Size
£819.00
- SKU:
- A0859-61Z3
- Purity:
- Purified by Protein G affinity chromatography.
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Mouse
- Immunogen:
- Recombinant protein corresponding to full length ADAMTS-13.
- Clone:
- 20A5
- Format:
- Supplied as as liquid in PBS, 0.1% BSA, 0.02% sodium azide.
- Extra Details:
- 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. Applications: Suitable for use in Immunofluorescence, ELISA and Western Blot. Other applications have not been tested. Recommended Dilutions: 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 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
- Shipping Conditions:
- Blue Ice