ADAM Metallopeptidase With Thrombospondin Type 1 Motif 13 / vWFCP (ADAMTS13) Antibody
221€ (100 µl)
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935106861
info@markelab.com
name
ADAM Metallopeptidase With Thrombospondin Type 1 Motif 13 / vWFCP (ADAMTS13) Antibody
category
Primary Antibodies
provider
Abbexa
reference
abx130605
tested applications
WB, IHC, IF/ICC
Description
vWFCP Antibody is a Rabbit Polyclonal against vWFCP.
Documents del producto
Instrucciones
Data sheet
Product specifications
| Category | Primary Antibodies |
| Immunogen Target | ADAM Metallopeptidase With Thrombospondin Type 1 Motif 13 / vWFCP (ADAMTS13) |
| Host | Rabbit |
| Reactivity | Human |
| Recommended Dilution | WB: 0.01-2 µg/ml, IHC: 5-20 µg/ml, IF/ICC: 5-20 µg/ml. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Purification | Purified by antigen-specific affinity chromatography, followed by Protein A affinity chromatography. |
| Size 1 | 100 µl |
| Size 2 | 200 µl |
| Size 3 | 1 ml |
| Form | Liquid |
| Tested Applications | WB, IHC, IF/ICC |
| Buffer | 0.01 M PBS, pH 7.4, containing 0.05% Proclin-300, 50% glycerol. |
| Availability | Shipped within 5-7 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | Q76LX8 |
| Alias | VWF-CP,VWFCP,Von Willebrand Factor-Cleaving Protease,ADAM-TS13,ADAMTS-13,A Disintegrin And Metalloproteinase With Thrombospondin Motifs 13 |
| Background | Antibody anti-ADAMTS13 |
| Status | RUO |
Descripción
ADAMTS13 (ADAM metallopeptidase with thrombospondin type 1 motif 13) is a member of the ADAMTS (a disintegrin and metalloproteinase with thrombospondin motifs) protein family. ADAMTS13 contains multiple domains, including a signal peptide, a propeptide region, a metalloproteinase domain, a disintegrin-like domain, and multiple thrombospondin type 1 (TSP1) motifs. ADAMTS13 is primarily known for its role in cleaving VWF. VWF normally forms long multimeric strands, which are essential for platelet adhesion and aggregation during clot formation. However, excessively large VWF multimers can lead to thrombotic disorders. ADAMTS13 cleaves these large VWF multimers into smaller, less active forms, thereby regulating platelet aggregation and preventing excessive clot formation. Deficiency or dysfunction of ADAMTS13 can result in a rare but severe condition called thrombotic thrombocytopenic purpura (TTP). In TTP, the lack of functional ADAMTS13 leads to the buildup of large VWF multimers, which can cause widespread microvascular thrombosis and thrombocytopenia. ADAMTS13 may also have other physiological functions that are not fully understood. Some studies suggest potential involvement in angiogenesis, inflammation, and tissue remodeling. Autoantibodies inhibit ADAMTS13 activity, while in hereditary TTP, genetic mutations affect the production or function of ADAMTS13. Both conditions lead to the accumulation of large VWF multimers, resulting in microvascular thrombosis and associated complications. ADAMTS13 activity has also been implicated in other thrombotic disorders, such as arterial thrombosis and stroke.
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