Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Species
      Human
    • Sequence
      MAAWFPDGIHTDSSTYRIVPGGYAVVGAAALAGAVTHTVSTAVIVFELTG QIAHILPVMIVVILANAVAQSLQPSLYDSIIRIKKLPYLPELGWGRHQQY RVRVEDIMVRDVPHVALSCTFRDLRLALHRTKGRMLALVESPESMILLGS IERSQVVALLGAQLSPARRRQHMQERRATQTSPLSDQEGPPTPEASVCFQ VNTEDSAFPAARGETHKPLKPALKRGPSVTRNLGESPTGSAESAGIALRS LFCGSPPPEAASEKLESCEKRKLKRVRISLASDADLEGEMSPEETHTIFS LLGVDHAYVTSIGRLIGIVTLKELRKAIEGSVTAQGVKVRPPLASFRDSA TSSSDTETTEVHALWGPHSRHGLPREGSPSDSDDKCQ
    • Amino acids
      1 to 387
    • Tags
      proprietary tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab158133 in the following tested applications.

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

  • Applications

    Western blot

    ELISA

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
  • 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
    • 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

Images

  • ab158133 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab158133 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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