ADAMTS-13, Human Antibody from HYCULT BIOTECH

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Antigenic SpecificityADAMTS-13, Human
Clone20A5
Host SpeciesMouse
Reactive Specieshuman
IsotypeIgG1
Formatunconjugated
Size100 µg
Concentrationn/a
ApplicationsImmuno assays, Immuno fluorescence, Western blot. RUO
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DescriptionThe monoclonal antibody 20A5 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 20A5 recognizes the central to C-terminal TSP-1 repeats 2 to 5 of ADAMTS-13 (amino acid 686-894).
Immunogenn/a
Other NamesVon Willebrand Factor-Cleaving Protease (VWFCP)
Gene, Accession #n/a
Catalog #HM2226-100UG
Price$488
Order / More InfoADAMTS-13, Human Antibody from HYCULT BIOTECH
Product Specific Referencesn/a
HYCULT BIOTECH
HYCULT BIOTECH
HYCULT BIOTECH
Frontstraat 2a
5405 PB Uden THE NETHERLANDS
P: +31 (0)413 251335
F: +31 (0)413 248353

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