Overview

Description

  • Nature

    Recombinant
  • Source

    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species

      Human
    • Sequence

      MGGCAGSRRRFSDSEGEETVPEPRLPLLDHQGAHWKNAVGFWLLGLCNNF SYVVMLSAAHDILSHKRTSGNQSHVDPGPTPIPHNSSSRFDCNSVSTAAV LLADILPTLVIKLLAPLGLHLLPYSPRVLVSGICAAGSFVLVAFSHSVGT SLCGVVFASISSGLGEVTFLSLTAFYPRAVISWWSSGTGGAGLLGALSYL GLTQAGLSPQQTLLSMLGIPALLLASYFLLLTSPEAQDPGGEEEAESAAR QPLIRTEAPESKPGSSSSLSLRERWTVFKGLLWYIVPLVVVYFAEYFINQ GLFELLFFWNTSLSHAQQYRWYQMLYQAGVFASRSSLRCCRIRFTWALAL LQCLNLVFLLADVWFGFLPSIYLVFLIILYEGLLGGAAYVNTFHNIALET SDEHREFAMAATCISDTLGISLSGLLALPLHDFLCQLS
    • Molecular weight

      74 kDa including tags
    • Amino acids

      1 to 438

Specifications

Our Abpromise guarantee covers the use of ab132559 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

  • Form

    Liquid
  • Additional notes

    Protein concentration is above or equal to 0.05 µg/µl.
  • 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: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names

    • Batten disease protein
    • Battenin
    • BTS
    • Ceroid lipofuscinosis neuronal 3
    • Ceroid lipofuscinosis neuronal 3 juvenile
    • Ceroid lipofuscinosis neuronal 3 juvenile (Batten Spielmeyer Vogt disease)
    • CLN 3
    • CLN3
    • CLN3_HUMAN
    • JNCL
    • MGC102840
    • Protein CLN3
    see all
  • Function

    Involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes.
  • Involvement in disease

    Defects in CLN3 are the cause of neuronal ceroid lipofuscinosis type 3 (CLN3) [MIM:204200]; also known as Batten disease. A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The hallmark of CLN3 is the ultrastructural pattern of lipopigment with a fingerprint profile, which can have 3 different appearances: pure within a lysosomal residual body; in conjunction with curvilinear or rectilinear profiles; and as a small component within large membrane-bound lysosomal vacuoles. The combination of fingerprint profiles within lysosomal vacuoles is a regular feature of blood lymphocytes from patients with CLN3.
  • Sequence similarities

    Belongs to the battenin family.
  • Post-translational
    modifications

    Highly glycosylated.
    Farnesylation is important for trafficking to lysosomes.
  • Cellular localization

    Lysosome membrane. Late endosome.
  • Information by UniProt

Images

  • 12.5% SDS-PAGE analysis of ab132559 stained with Coomassie Blue.

References

ab132559 has not yet been referenced specifically in any publications.

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