ADAMTS13 Antibody (5C11)

Product Discontinued

ADAMTS13 Antibody (5C11) Summary

• Immunogen: The monoclonal antibody 5C11 recognizes the central to C-terminal TSP-1 repeats 2 to 5 of ADAMTS-13 (amino acid 686-894).
• Isotype: IgG1
• Clonality: Monoclonal
• Host: Mouse
• Gene: ADAMTS13
• Purity: Protein A or G purified

Applications/Dilutions

• Dilutions:
  • Immunoassay
  • Western Blot 1:100-1:2000
• Application Notes: This antibody works in Immunoassay, Western Blot (non-reducing conditions)

Reactivity Notes

Human

Packaging, Storage & Formulations

• Storage: Store at 4C. Do not freeze.
• Buffer: PBS with 1% BSA
• Preservative: 0.02% Sodium Azide
• Purity: Protein A or G purified

Alternate Names for ADAMTS13 Antibody (5C11)

• A disintegrin and metalloproteinase with thrombospondin motifs 13
• a disintegrin-like and metalloprotease (reprolysin type) with thrombospondintype 1 motif, 13
• ADAM metallopeptidase with thrombospondin type 1 motif, 13
• ADAMTS13
• ADAM-TS13
• ADAMTS-13
• DKFZp434C2322
• EC 3.4.24.14
• EC 3.4.24.82
• EC 3.4.24.87
• FLJ42993
• MGC118899
• MGC118900
• TTP
• TTPADAM-TS 13
• vWF-cleaving protease
• vWF-CP
• vWF-CPC9orf8
• VWFCPvon Willebrand factor-cleaving protease

Background

The monoclonal antibody 5C11 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 ug/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.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Bioinformatics

• Gene Symbol: ADAMTS13