Overview

Properties

Applications

Our Abpromise guarantee covers the use of ab28275 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Notes
WB
  • Application notesWB: 1/1000 - 1/5000. Detects a band of approximately 190 kDa (predicted molecular weight: 154 kDa). Glycosylation and the abundance of cysteine residues gives ADAMTS-13 an apparent molecular weight of 190 kDa on reduced SDS PAGE gels. Higher concentrations of antibody may be needed for samples from more distantly related species. Dilution optimised using Chromogenic detection. Not yet tested in other applications. Optimal dilutions/concentrations should be determined by the end user.
  • Target

    • FunctionCleaves the vWF multimers in plasma into smaller forms.
    • Tissue specificityPlasma. Expressed primarily in liver.
    • Involvement in diseaseDefects in ADAMTS13 are the cause of thrombotic thrombocytopenic purpura congenital (TTP) [MIM:274150]; also known as Upshaw-Schulman syndrome (USS). A hematologic disease characterized by hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decreased renal function and fever.
    • Sequence similaritiesContains 2 CUB domains.
      Contains 1 disintegrin domain.
      Contains 1 peptidase M12B domain.
      Contains 8 TSP type-1 domains.
    • DomainThe pro-domain is not required for folding or secretion and does not perform the common function of maintening enzyme latency.
      The spacer domain is necessary to recognize and cleave vWF. The C-terminal TSP type-1 and CUB domains may modulate this interaction.
    • Post-translational
      modifications
      May contain a C-mannosylation site and O-fucosylation sites in the TSP type-1 domains.
      The precursor is processed by a furin endopeptidase which cleaves off the pro-domain.
    • Cellular localizationSecreted.
    • Target information above from: UniProt accession Q76LX8 The UniProt Consortium
      The Universal Protein Resource (UniProt) in 2010
      Nucleic Acids Res. 38:D142-D148 (2010) .

      Information by UniProt
    • Database links
    • Alternative names
      • A disintegrin and metalloproteinase with thrombospondin motifs 13 antibody
      • A disintegrin like and metalloprotease (reprolysin type) with thrombospondin type 1 motif 13 antibody
      • A disintegrin like and metalloprotease with thrombospondin type 1 motif 13 antibody
      • A disintegrin-like and metalloprotease (Reprolysin type) with thrombospondin type 1 motif 13 antibody
      • ADAM metallopeptidase with thrombospondin type 1 motif 13 antibody
      • ADAM TS antibody
      • ADAM-TS 13 antibody
      • ADAM-TS13 antibody
      • ADAMTS 13 antibody
      • ADAMTS-13 antibody
      • ADAMTS13 antibody
      • ADAMTS13 protein antibody
      • ATS13_HUMAN antibody
      • C9orf8 antibody
      • EC 3.4.24.- antibody
      • TTP antibody
      • Von Willebrand factor cleaving protease antibody
      • Von Willebrand factor cleaving protease antibody
      • von Willebrand factor-cleaving protease antibody
      • vWF cleaving protease antibody
      • vWF CP antibody
      • vWF-cleaving protease antibody
      • vWF-CP antibody
      • vWFCP antibody
      see all

    Anti-ADAMTS13 antibody images

    • All lanes : Anti-ADAMTS13 antibody (ab28275) at 1 µg/ml

      Lane 1 : Recombinant Human ATS-13 at 0.08 µg
      Lane 2 : Recombinant Human ATS-13 at 0.04 µg
      Lane 3 : Recombinant Human ATS-13 at 0.02 µg


      Predicted band size : 154 kDa

    References for Anti-ADAMTS13 antibody (ab28275)

    ab28275 has not yet been referenced specifically in any publications.

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