The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/1000 - 1/5000. Detects a band of approximately 190 kDa (predicted molecular weight: 154 kDa).Can be blocked with ADAMTS13 peptide (ab41249). 1/1000 when using colorimetric substrates such as BCIP/NBT - 1/5000, when using chemiluminescent substrates. Glycosylation and the abundance of cysteine residues gives ADAMTS 13 an apparent molecular weight of 190 kDa on reduced SDS PAGE gels. Several bands at 110-190 kDa are observed on Western blots, possibly indicating differentially processed ADAMTS 13. Dilution optimised using Chromogenic detection.
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.