Description

Associated products

Specifications

Our Abpromise guarantee covers the use of ab218163 in the following tested applications.

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

  • Applications

    Blocking - Blocking peptide for Anti-ClC-2 antibody [EPR6492(2)] (ab154798)

  • Form

    Lyophilised
  • Additional notes

    Reconstitution instructions:

    - 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...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Store at -20°C.

General Info

  • Alternative names

    • Chloride Channel 2
    • Chloride channel protein 2
    • Chloride channel, voltage sensitive 2
    • CIC 2
    • CIC2
    • ClC-2
    • CLC2
    • Clcn2
    • CLCN2_HUMAN
    • ECA2
    • ECA3
    • EG13
    • EGI11
    • EGMA
    • EJM6
    • EJM8
    • PKA-activated chloride channel
    see all
  • Function

    Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport.
  • Tissue specificity

    Ubiquitously expressed. Moderately expressed in aortic and coronary vascular smooth muscle cells and expressed at a low level in aortic endothelial cells.
  • Involvement in disease

    Defects in CLCN2 are associated with susceptibility to idiopathic generalized epilepsy type 11 (IGE11) [MIM:607628]. A disorder characterized by recurring generalized seizures in the absence of detectable brain lesions and/or metabolic abnormalities. Generalized seizures arise diffusely and simultaneously from both hemispheres of the brain.
    Defects in CLCN2 are the cause of childhood absence epilepsy type 3 (ECA3) [MIM:607682]. ECA3 is a subtype of idiopathic generalized epilepsy (IGE) characterized by onset at age 6-7 years, frequent absence seizures (several per day) and bilateral, synchronous, symmetric 3 Hz spike waves on EEG. During adolescence, tonic-clonic and myoclonic seizures develop.
    Defects in CLCN2 are associated with juvenile absence epilepsy type 2 (JAE2) [MIM:607628]. JAE is a subtype of idiopathic generalized epilepsy (IGE) characterized by onset occurring around puberty, absence seizures, generalized tonic-clonic seizures (GTCS), GTCS on awakening and myoclonic seizures.
    Defects in CLCN2 are associated with juvenile myoclonic epilepsy type 8 (EJM8) [MIM:607628]. A subtype of idiopathic generalized epilepsy. Patients have afebrile seizures only, with onset in adolescence (rather than in childhood) and myoclonic jerks which usually occur after awakening and are triggered by sleep deprivation and fatigue.
  • Sequence similarities

    Belongs to the chloride channel (TC 2.A.49) family. ClC-2/CLCN2 subfamily.
    Contains 2 CBS domains.
  • Cellular localization

    Membrane.
  • Information by UniProt

References

ab218163 has not yet been referenced specifically in any publications.

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