Recombinant Human ADAMTS13 protein (ab112399)

Overview

Description

  • NatureRecombinant
  • SourceWheat germ
  • Amino Acid Sequence
    • AccessionQ76LX8
    • SpeciesHuman
    • SequenceFINVAPHARIAIHALATNMGAGTEGANASYILIRDTHSLRTTAFHGQQVL YWESESSQAEMEFSEGFLKAQASLRGQYWTLQSWVPEMQDPQSWKGKEGT
    • Molecular weight37 kDa including tags
    • Amino acids1328 to 1427

Specifications

Our Abpromise guarantee covers the use of ab112399 in the following tested applications.

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

  • Applications

    ELISA

    SDS-PAGE

    Western blot

    Other

  • FormLiquid
  • Additional notesBest use within three months from the date of receipt of this protein.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.79% Tris HCl, 0.31% Glutathione

General Info

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

Recombinant Human ADAMTS13 protein images

  • 12.5% SDS-PAGE analysis of ab112399. Stained with Coomassie Blue

References for Recombinant Human ADAMTS13 protein (ab112399)

ab112399 has not yet been referenced specifically in any publications.

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