murine MAb against human ADAMTS13, clone 6.1
Description
ADAMTS13, also known as von Willebrand Factor (vWF) cleaving protease, is a zinc metalloproteinase that cleaves ultra large vWF multimers (UL-vWF) at the Tyr(1605) - Met(1606) bond located in the A2 region of vWF.(1) Studies have shown that low levels of ADAMTS13 activity are associated with Thrombotic Thrombocytopenia Purpura (TTP), a life-threatening hematological condition characterized by low platelet count, microvascular thrombi, red cell fragmentation, CNS and renal complications.(2,3) A deficiency or low level of ADAMTS13 activity (<5%) may lead to an accumulation of UL-vWF multimers.(4) The UL-vWF multimers will bind to receptors on platelets inducing platelet aggregation and formation of intravascular thrombi.
Preparation
The monoclonal antibodies are directed against human ADAMTS13, von Willebrand cleaving protease. Mice were immunized with purified recombinant full-length human ADAMTS13 (200 kDa) expressed in a eukaryotic cell line. The antibodies have been purified from cell culture supernatant using Protein G affinity chromatography.
Presentation
Screw capped vial containing 100 µg of purified antibody in PBS pH 7.4, 0.01 % ProClin. The IgG concentration is 1 mg/ml. Spin the vial briefly before opening.
Applications
Storage and Stability
Store the antibody at 2°-8°C. For long-term storage the antibody should be aliquoted and stored at
–20°C or colder. It is recommended to avoid freeze-thaw cycles.
Category: Research use only
Type: Antibody
Product Availability: Worldwide
Manufacturer: ImmBioMed GmbH & Co KG, Germany
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murine MAb against human ADAMTS13, clone 6.1
Cat.No. ADG3306
Artikelnr.: 938619
Einheit: 100 µg
Code: ADG3306
Hersteller: ImmBioMed GmbH & Co. KG
References
- Furlan, M., Robles, R. and Lämmle, B. L. Partial Purification and Characterization of a Protease from Human Plasma Cleaving von Willebrand Factor to Fragments Produced by In Vivo Proteolysis. Blood 1996, 87(10): 4223-4234.
- Tsai, H. M. Physiologic Cleavage of von Willebrand Factor by a Plasma Protein Is Dependent on Its Conformation and Requires Calcium Ion. Blood 1996, 87(10): 4235-4244.
- Furlan, M., et al. Von Willebrand Factor-Cleaving Protease In Thrombotic Thrombocytopenic Purpura And The Hemolytic-Uremic Syndrome. The New England Journal of Medicine 1998, 339(22): 1578-1584.
- Moake, J. L., et al. Unusually large plasma factor VIII:von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. The New England Journal of Medicine 1982, 307(23): 1432-1435.