ADAMTS-13, Human, mAb 5C11
Catalog #: HM2225
Quantity: 100 µg
The monoclonal antibody 5C11 recognizes human ADAMTS-13, A Disintegrin And Metalloprotease with ThromboSpondin type 1 domain 13. ADAMTS-13 is produced by hepatic stellate cells and in smaller amounts by human endothelial cells, and is present in plasma at a concentration of approximately 1 ug/ml. 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 190 kDa 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 140 kDa, 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. The monoclonal antibody 5C11 recognizes the central to C-terminal TSP-1 repeats 2 to 5 of ADAMTS-13 (amino acid 686-894).
|Product type||Monoclonal antibodies|
|Formulation||1 ml (100 µg/ml) 0.2 µm filtered antibody solution in PBS, containing 0.1% bovine serum albumin and 0.02% sodium azide.|
|Alias||Von Willebrand Factor-Cleaving Protease (VWFCP)|
|Storage and stability||Product should be stored at 4 °C. Under recommended storage conditions, product is stable for one year.|
|Precautions||For research use only. Not for use in or on humans or animals or for diagnostics. It is the responsibility of the user to comply with all local/state and Federal rules in the use of this product. Hycult Biotech is not responsible for any patent infringements that might result with the use or derivation of this product.|
|Disease||Cardiology and metabolism|
Use:For Western blotting dilutions to be used depend on detection system applied. It is recommended that users test the reagent and determine their own optimal dilutions. The typical starting working dilution is 1:50.