The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Best use within three months from the date of receipt of this protein.
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Preparation and Storage
Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.79% Tris HCl, 0.31% Glutathione
A disintegrin and metalloproteinase with thrombospondin motifs 13
A disintegrin like and metalloprotease (reprolysin type) with thrombospondin type 1 motif 13
A disintegrin like and metalloprotease with thrombospondin type 1 motif 13
ADAM metallopeptidase with thrombospondin type 1 motif 13
Von Willebrand factor cleaving protease
von Willebrand factor-cleaving protease
vWF cleaving protease
Cleaves the vWF multimers in plasma into smaller forms.
Plasma. Expressed primarily in liver.
Involvement in disease
Defects in ADAMTS13 are the cause of thrombotic thrombocytopenic purpura congenital (TTP) [MIM:274150]; also known as Upshaw-Schulman syndrome (USS). A hematologic disease characterized by hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decreased renal function and fever.
The pro-domain is not required for folding or secretion and does not perform the common function of maintening enzyme latency. The spacer domain is necessary to recognize and cleave vWF. The C-terminal TSP type-1 and CUB domains may modulate this interaction.
May contain a C-mannosylation site and O-fucosylation sites in the TSP type-1 domains. The precursor is processed by a furin endopeptidase which cleaves off the pro-domain.