Overview

  • Product name
    CLN8 peptide

Description

  • Nature
    Synthetic

Specifications

Our Abpromise guarantee covers the use of ab193322 in the following tested applications.

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

  • Applications

    Blocking

  • Form
    Liquid
  • Additional notes

    - 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.

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at -20°C or -80°C.

General Info

  • Alternative names
    • C8orf61
    • Ceroid-lipofuscinosis, neuronal 8 (epilepsy, progressive with mental retardation)
    • Cln8
    • CLN8 gene
    • CLN8_HUMAN
    • EPMR
    • Protein CLN8
    see all
  • Function
    Could play a role in cell proliferation during neuronal differentiation and in protection against cell death.
  • Involvement in disease
    Defects in CLN8 are the cause of neuronal ceroid lipofuscinosis type 8 (CLN8) [MIM:600143]. A form of neuronal ceroid lipofuscinosis with onset in childhood. 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 lipopigment patterns observed most often in neuronal ceroid lipofuscinosis type 8 comprise mixed combinations of granular, curvilinear, and fingerprint profiles.
    Defects in CLN8 are the cause of neuronal ceroid lipofuscinosis type 8 Northern epilepsy variant (CLN8NE) [MIM:610003]. A form of neuronal ceroid lipofuscinosis clinically characterized by epilepsy that presents between 5 and 10 years of age with frequent tonic-clonic seizures followed by progressive mental retardation. Visual loss is not a prominent feature. Intracellular accumulation of autofluorescent material results in curvilinear and granular profiles on ultrastructural analysis.
  • Sequence similarities
    Contains 1 TLC (TRAM/LAG1/CLN8) domain.
  • Post-translational
    modifications
    Does not seem to be N-glycosylated.
  • Cellular localization
    Endoplasmic reticulum membrane. Endoplasmic reticulum-Golgi intermediate compartment membrane.
  • Information by UniProt

References

ab193322 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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