ADAMTS13 anticorps

N° du produit ABIN2192078

Détail du produit anti-ADAMTS13 anticorps

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

Reactivité: Humain

Hôte: Souris

Clonalité: Monoclonal

Conjugué: Cet anticorp ADAMTS13 est non-conjugé

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

Stérilité: 0.2 μm filtered

Clone: 20A5

Information d'application

Indications d'application: 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

Stockage

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

Agent conservateur: Sodium azide

Précaution d'utilisation: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Stock: 4 °C

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

Date de péremption: 12 months

Détails sur ADAMTS13

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

Autre désignation: Adamts-13 (ADAMTS13 Produits)

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

Sujet: 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)