Overview

  • Product name

    Recombinant Human CLCN1 protein
  • Protein length

    Full length protein

Description

  • Nature

    Recombinant
  • Source

    Wheat germ
  • Amino Acid Sequence
    • Species

      Human
    • Sequence

      MEQSRSQQRGGEQSWWGSDPQYQYMPFEHCTSYGLPSENGGLQHRLRKDA GPRHNVHPTQIYGHHKEQFSDREQDIGMPKKTGSSSTVDSKDEDHYSKCQ DCIHRLGQVVRRKLGEDGIFLVLLGLLMALVSWSMDYVSAKSLQAYKWSY AQMQPSLPLQFLVWVTFPLVLILFSALFCHLISPQAVGSGIPEMKTILRG VVLKEYLTMKAFVAKVVALTAGLGSGIPVGKEGPFVHIASICAAVLSKFM SVFCGVYEQPYYYSDILTVGCAVGVGCCFGTPLGGVLFSIEVTSTYFAVR NYWRGFFAATFSAFVFRVLAVWNKDAVTITALFRTNFRMDFPFDLKELPA FAAIGICCGLLGAVFVYLHRQVMLGVRKHKALSQFLAKHRLLYPGIVTFV IASFTFPPGMGQFMAGELMPREAISTLFDNNTWVKHAGDPESLGQSAVWI HPRVNVVIIIFLFFVMKFWMSIVATTMPIPCGGFMPVFVLGAAFGRLVGE IMAMLFPDGILFDDIIYKILPGGYAVIGAAALTGAVSHTVSTAVICFELT GQIAHILPMMVAVILANMVAQSLQPSLYDSIIQVKKLPYLPDLGWNQLSK YTIFVEDIMVRDVKFVSASYTYGELRTLLQTTTVKTLPLVDSKDSMILLG SVERSELQALLQRHLCPERRLRAAQEMARKLSELPYDGKARLAGEGLPGA PPGRPESFAFVDEDEDEDLSGKSELPPSLALHPSTTAPLSPEEPNGPLPG HKQQPEAPEPAGQRPSIFQSLLHCLLGRARPTKKKTTQDSTDLVDNMSPE EIEAWEQEQLSQPVCFDSCCIDQSPFQLVEQTTLHKTHTLFSLLGLHLAY VTSMGKLRGVLALEELQKAIEGHTKSGVQLRPPLASFRNTTSTRKSTGAP PSSAENWNLPEDRPGATGTGDVIAASPETPVPSPSPEPPLSLAPGKVEGE LEELELVESPGLEEELADILQGPSLRSTDEEDEDELIL
    • Amino acids

      1 to 988
    • Tags

      GST tag N-Terminus

Specifications

Our Abpromise guarantee covers the use of ab158131 in the following tested applications.

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

  • Applications

    ELISA

    Western blot

  • 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 protein
    • Chloride channel protein 1
    • Chloride channel protein skeletal muscle
    • CIC 1
    • CIC1
    • ClC-1
    • CLC1
    • Clcn1
    • CLCN1_HUMAN
    • MGC138361
    • MGC142055
    • skeletal muscle
    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

    Predominantly expressed in skeletal muscles.
  • Involvement in disease

    Defects in CLCN1 are the cause of Thomsen disease (THD) [MIM:160800]; also known as autosomal dominant myotonia congenita (MCD). THD is characterized by skeletal muscle stiffness (delayed relaxation), due to membrane hyperexcitability. A variant form of Thomsen disease is myotonia levior that is characterized by milder symptoms, later onset and absence of muscle hypo- and hypertrophy.
    Defects in CLCN1 are the cause of autosomal recessive myotonia congenita (MCR) [MIM:255700]; also known as Becker disease.
  • Sequence similarities

    Belongs to the chloride channel (TC 2.A.49) family. ClC-1/CLCN1 subfamily.
    Contains 2 CBS domains.
  • Cellular localization

    Membrane.
  • Information by UniProt

Images

  • ab158131 on a 12.5% SDS-PAGE stained with Coomassie Blue.

References

ab158131 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab158131.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
For licensing inquiries, please contact partnerships@abcam.com

Sign up