Human ADAMTS13 ELISA Kit (ab213752)
Key features and details
- Sensitivity: 20 pg/ml
- Range: 0.78 ng/ml - 50 ng/ml
- Sample type: Cell culture supernatant, Cit plasma, EDTA Plasma, Hep Plasma, Serum
- Detection method: Colorimetric
- Assay type: Sandwich (quantitative)
- Reacts with: Human
Properties
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Storage instructions
Store at -20°C. Please refer to protocols. -
Components Identifier 1 x 96 tests ABC Diluent Buffer Blue Cap 1 x 12ml Adhesive Plate Seal 4 units Antibody Diluent Buffer Green Cap 1 x 12ml Anti-Human ADAMTS13 coated Microplate (12 x 8 wells) 1 unit Avidin-Biotin-Peroxidase Complex (ABC) 1 x 100µl Biotinylated anti- Human ADAMTS13 antibody 1 x 130µl Lyophilized recombinant Human ADAMTS13 standard 2 vials Sample Diluent Buffer Green Cap 1 x 30ml TMB Color Developing Agent Black Cap 1 x 10ml TMB Stop Solution Yellow Cap 1 x 10ml -
Research areas
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Function
Cleaves the vWF multimers in plasma into smaller forms. -
Tissue specificity
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. -
Sequence similarities
Contains 2 CUB domains.
Contains 1 disintegrin domain.
Contains 1 peptidase M12B domain.
Contains 8 TSP type-1 domains. -
Domain
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. -
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. -
Cellular localization
Secreted. - Information by UniProt
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Alternative names
- 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
see all -
Database links
- Entrez Gene: 11093 Human
- Omim: 604134 Human
- SwissProt: Q76LX8 Human
- Unigene: 131433 Human
Images
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Human ADAMTS13 ELISA Kit (ab213752) Standard Curve.
Human ADAMTS13 ELISA Kit (ab213752) Standard Curve.
