The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use at 1-5 µg/mg of lysate.
应用说明Is unsuitable for WB.
功能Cleaves the vWF multimers in plasma into smaller forms.
组织特异性Plasma. Expressed primarily in liver.
疾病相关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.
结构域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.
翻译后修饰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.