ADAMTS13 antibody (AA 686-894)

Catalog No. ABIN1105249

Product Details anti-ADAMTS13 Antibody

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

Binding Specificity: AA 686-894

Reactivity: Human

Host: Mouse

Clonality: Monoclonal

Conjugate: This ADAMTS13 antibody is un-conjugated

Application: Western Blotting (WB), Immunofluorescence (IF), Enzyme Immunoassay (EIA)

Cross-Reactivity (Details): Species reactivity (tested):Human

Purification: Protein G

Clone: 20A5

Isotype: IgG1

Application Details

Application Notes: Optimal working dilution should be determined by the investigator.

Restrictions: For Research Use only

Handling

Concentration: 0.1 mg/mL

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

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: Store at 2 - 8 °C.

Target Details for ADAMTS13

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

Alternative Name: ADAMTS13 (ADAMTS13 Products)

Background: 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.Synonyms: A disintegrin and metalloproteinase with thrombospondin motifs 13, ADAM-TS13, ADAMTS-13, C9orf8, vWF-cleaving protease, von Willebrand factor-cleaving protease

UniProt: Q76LX8