WB, Flow Cytmore details Unsuitable for:
ICC,IHC-P or IP
Human Does not react with:
Synthetic peptide (the amino acid sequence is considered to be commercially sensitive) within Human ADAMTS13 aa 50-150 (Cysteine residue). The exact sequence is proprietary. Database link: Q76LX8
Human fetal liver, A549 and HepG2 cell lysates; A549 cells.
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents
We are constantly working hard to ensure we provide our customers with best in class antibodies. As a result of this work we are pleased to now offer this antibody in purified format. We are in the process of updating our datasheets. The purified format is designated 'PUR' on our product labels. If you have any questions regarding this update, please contact our Scientific Support team.
This product is a recombinant rabbit monoclonal antibody.
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
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).
1/100 - 1/500.
ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody.
Is unsuitable for ICC,IHC-P or IP.
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.