The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use at an assay dependent dilution.
1/50. Use under non reducing condition. Predicted molecular weight: 154 kDa.
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.