Product nameADAMTS13 peptide
See all ADAMTS13 proteins and peptides
Our Abpromise guarantee covers the use of ab41249 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Blocking - Blocking peptide for Anti-ADAMTS13 antibody (ab28274)
Purity> 95 % SDS-PAGE.
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Preparation and Storage
Stability and Storage
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
Constituents: 0.001% Tween, 0.714% HEPES, 0.0584% EDTA, 0.87% Sodium chloride
- A disintegrin and metalloproteinase with thrombospondin motifs 13
- A disintegrin like and metalloprotease (reprolysin type) with thrombospondin type 1 motif 13
- A disintegrin like and metalloprotease with thrombospondin type 1 motif 13
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.
Sequence similaritiesContains 2 CUB domains.
Contains 1 disintegrin domain.
Contains 1 peptidase M12B domain.
Contains 8 TSP type-1 domains.
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.
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.
- Information by UniProt