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You are here:Home » Antibodies » Abs to Matrix Metalloproteinases » Anti -ADAMTS-13 (Von Willebrand Factor-Cleaving Protease (VWFCP))

Anti -ADAMTS-13 (Von Willebrand Factor-Cleaving Protease (VWFCP))

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Specifications

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
ApplicationsSuitable 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 StabilityMay 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.
Clone TypeMonoclonal
IsotypeIgG1
Clone No20A5
HostMouse
Concentration~0.1mg/ml
FormSupplied as as liquid in PBS, 0.1% BSA, 0.02% sodium azide.
PurityPurified by Protein G affinity chromatography.
ImmunogenRecombinant corresponding to full length ADAMTS-13.
SpecificityRecognizes ADAMTS-13. Reacts with the central to C-terminal TSP-1 repeats 2-5 of ADAMTS-13 (aa686-894). Species Crossreactivity: Human
Important NoteThis 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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