The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Application notesWB: 1/1000 - 1/5000. Detects a band of approximately 190 kDa (predicted molecular weight: 154 kDa). Glycosylation and the abundance of cysteine residues gives ADAMTS-13 an apparent molecular weight of 190 kDa on reduced SDS PAGE gels. Higher concentrations of antibody may be needed for samples from more distantly related species. Dilution optimised using Chromogenic detection. Not yet tested in other applications. Optimal dilutions/concentrations should be determined by the end user.
FunctionCleaves the vWF multimers in plasma into smaller forms.
Tissue specificityPlasma. Expressed primarily in liver.
Involvement in diseaseDefects 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.
DomainThe 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.
Post-translational modificationsMay 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.