ADAMTS13 Antibody

Immunoprecipitation: ADAMTS13 Antibody [NB100-55261] - Detection of Human ADAMTS13 on HeLa whole cell lysates using NB100-55259, NB100-55260, NB100-55261 and NB100-55262. For blotting IPed ADAMTS13, goat anti-ADAMTS13 antibody was used.

• Reactivity: Hu
• Applications: WB, IP
• Clonality: Polyclonal
• Host: Goat
• Conjugate: Unconjugated
• Concentration: 1.0 mg/ml

ADAMTS13 Antibody Summary

• Immunogen: The immunogen recognized by this antibody maps to a region between residue 825 and 875 of human ADAM (a disintegrin-like and metalloprotease) metallopeptidase with thrombospondin type 1 motif, 13 using the numbering given in entry NP_620594.1 (GeneID 1109
• Isotype: IgG
• Clonality: Polyclonal
• Host: Goat
• Gene: ADAMTS13
• Purity: Immunogen affinity purified

Applications/Dilutions

• Dilutions:
  • Immunoprecipitation 1:10-1:500
  • Western Blot 1:1000-1:10000

Packaging, Storage & Formulations

• Storage: Store at 4C. Do not freeze.
• Buffer: Tris-Citrate/Phosphate (pH 7.0 - 8.0)
• Preservative: 0.09% Sodium Azide
• Concentration: 1.0 mg/ml
• Purity: Immunogen affinity purified

Alternate Names for ADAMTS13 Antibody

• 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

ADAMTS13 was first identified by its ability to cleave the ultra large aggregates of vWF into smaller forms in the serum. A thrombocytopenic disorder called thrombic thrombocytopenic purpura (TTR) was shown to be due to a deficiency in the cleavage of von Willebrands factor, and ADAMTS13 was identified as the enzyme responsible. ADAMTS13 cleaves the Tyr1605 to Met1606 bond in the A2 domain of vWF, a process stimulated by shear stress in the circulation and by binding to either platelet glycoprotein IBa or to heparin. The cleaved vWF leads to decreased platelet adhesion. An idiopathic form of TTR is found in patients with auto antibodies to ADAMTS13. The full length ADAMTS-1 sequence codes for a 1,427 amino acid protein, with a predicted mass of 153.6 kDa, but glycosylation and the abundance of cysteine residues gives ADAMTS-13 an apparent molecular weight of about 190 kDa on reduced SDS PAGE gels. The variants reported include 1,371, 1,340 and 344 amino acids, with predicted mass of 130.3, 144.5 and 36.7 kDa respectively. Many bands of varying sizes can be seen on Western blots, between 110-190 kDa, perhaps indicating differentially processed ADAMTS-13.

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.

Publications for ADAMTS13 Antibody (NB100-55261)(2)

Publications using NB100-55261

• Pikovsky O, Arafat M, Ovadia H, et al. Congenital thrombotic thrombocytopenic purpura in a large cohort of patients carrying a novel mutation in ADAMTS13 gene Thromb. Res. Dec 5 2019 [PMID: 31837614] (IP, WB, Human)
• Turner NA, Nolasco L, Ruggeri ZM et al. Endothelial cell ADAMTS-13 and VWF: production, release, and VWF string cleavage. Blood 2009 Dec [PMID: 19822897]

Bioinformatics

• Gene Symbol: ADAMTS13
• Entrez:
  • 11093(Human)
• Uniprot:
  • NP_620594.1(Human)