ADAMTS13 antibody

Catalog No. ABIN2192078

Product Details anti-ADAMTS13 Antibody

Target: ADAMTS13 (ADAM Metallopeptidase with Thrombospondin Type 1 Motif, 13 (ADAMTS13))

Reactivity: Human

Host: Mouse

Clonality: Monoclonal

Conjugate: This ADAMTS13 antibody is un-conjugated

Application: Western Blotting (WB), Immunofluorescence (IF), Immunoassay (IA)

Sterility: 0.2 μm filtered

Clone: 20A5

Application Details

Application Notes: For Western blotting and immunofluorescence dilutions to be used depend on detection system applied. It is recommended that users test the reagent and determine their own optimal dilutions. The typical starting working dilution is 1:50.

Restrictions: For Research Use only

Handling

Buffer: PBS, containing 0.1 % bovine serum albumin and 0.02 % sodium azide.

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: 4 °C

Storage Comment: Product should be stored at 4 °C. Under recommended storage conditions, product is stable for one year.

Expiry Date: 12 months

Target Details for ADAMTS13

Target: ADAMTS13 (ADAM Metallopeptidase with Thrombospondin Type 1 Motif, 13 (ADAMTS13))

Alternative Name: Adamts-13 (ADAMTS13 Products)

Synonyms: ADAMTS13 antibody, ADAM-TS13 antibody, ADAMTS-13 antibody, C9orf8 antibody, VWFCP antibody, vWF-CP antibody, Gm710 antibody, ADAM metallopeptidase with thrombospondin type 1 motif 13 antibody, a disintegrin-like and metallopeptidase (reprolysin type) with thrombospondin type 1 motif, 13 antibody, ADAM metallopeptidase with thrombospondin type 1 motif, 13 antibody, ADAMTS13 antibody, Adamts13 antibody, adamts13 antibody

Background: The monoclonal antibody 20A5 recognizes human ADAMTS-13, A Disintegrin And Metalloprotease with ThromboSpondin type 1 domain 13. ADAMTS-13 is produced by hepatic stellate cells and in smaller amounts by human endothelial cells, and is present in plasma at a concentration of approximately 1 μg/mL. ADAMTS-13 is a zinc-containing metalloprotease belonging to the ADAMTS family characterized by a protease domain, an adjacent disintegrin-like domain, one or more thrombospondin type 1 repeats, a cystein-rich domain and a typical spacer region. ADAMTS-13 is composed of a series of domains (amino to carboxy terminal): metalloprotease, disintegrin-like, central thrombospondin-1 (TSP-1), cysteine-rich, spacer, seven additional TSP-1 domains and two unique CUB domains. ADAMTS-13 has no hydrophobic transmembrane domain, and hence it is not anchored in the cell membrane. The apparent molecular weight is 170 or 190 kDa on non-reducing or reducing SDS-PAGE, respectively. ADAMTS-13 has an important function in haemostasis, where it catalyzes the cleavage of the peptide bond between tyrosine-1605 and methionine-1606 in the A2 domain of von Willebrand Factor (VWF), resulting in 2 electrophoretic reduced fragments of 176 and 140 kDa, respectively. This process renders large multimers less adhesive and hence less reactive in the setting of thrombus formation. ADAMTS-13 is therefore said to be a natural anti-thrombotic agent. Severe ADAMTS-13 deficiency is associated with systemic microvascular thrombosis in familial or acquired thrombotic thrombocytopenic purpura (TTP). The accumulation of non-cleaved large VWF multimers causes spontaneous systemic platelet aggregation blocking oxygen supply to vital organs. This life-threatening disorder can lead to ischemic disease with (multiple) organ failure. The monoclonal antibody 20A5 recognizes the central to C-terminal TSP-1 repeats 2 to 5 of ADAMTS- 13 (amino acid 686-894). Aliases Von Willebrand Factor-Cleaving Protease (VWFCP)