概述

描述

  • 性质Synthetic

技术指标

Our Abpromise guarantee covers the use of ab86257 in the following tested applications.

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

  • 纯度70 - 90% by HPLC.

  • 形式Liquid
  • 补充说明

    - First try to dissolve a small amount of peptide in either water or buffer. The more charged residues on a peptide, the more soluble it is in aqueous solutions.
    - If the peptide doesn’t dissolve try an organic solvent e.g. DMSO, then dilute using water or buffer.
    - Consider that any solvent used must be compatible with your assay. If a peptide does not dissolve and you need to recover it, lyophilise to remove the solvent.
    - Gentle warming and sonication can effectively aid peptide solubilisation. If the solution is cloudy or has gelled the peptide may be in suspension rather than solubilised.
    - Peptides containing cysteine are easily oxidised, so should be prepared in solution just prior to use.

  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    Information available upon request.

常规信息

  • 别名
    • 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
  • 功能Involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes.
  • 疾病相关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.
  • 序列相似性Belongs to the battenin family.
  • 翻译后修饰Highly glycosylated.
    Farnesylation is important for trafficking to lysosomes.
  • 细胞定位Lysosome membrane. Late endosome.
  • Information by UniProt

Human CLN3 peptide (ab86257)参考文献

ab86257 has not yet been referenced specifically in any publications.

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