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Chloride Channel 1, Skeletal Muscle (CLCN1) (N-Term) antibody

Details for Product No. ABIN350218, Supplier: Log in to see
  • CLCN1
  • si:dkey-14o18.5
  • Clc-1
  • Clc1
  • SMCC1
  • adr
  • mto
  • myotonia
  • nmf355
  • SMCC
  • CLC1
  • chloride channel 1, skeletal muscle
  • chloride channel 1, skeletal muscle (Thomsen disease, autosomal dominant)
  • chloride channel 1a
  • similar to chloride channel 1, skeletal muscle (Thomsen disease, autosomal dominant)
  • chloride channel 1
  • chloride channel, voltage-sensitive 1
  • CLCN1
  • clcn1a
  • Clcn1
Immunohistochemistry (IHC), Western Blotting (WB)
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Immunogen A synthetic peptide from the n-terminal region of human Chloride channel protein 1 (CLCN1) conjugated to an immunogenic carrier protein was used as the antigen.
Specificity Specific for CLCN1.
Purification Whole serum
Alternative Name CLCN1 (CLCN1 Antibody Abstract)
Background Function: Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume, membrane potential stabilization, signal transduction and transepithelial transport. The CLC channel family contains both chloride channels and proton-coupled anion transporters that exchange chloride or another anion for protons. The absence of conserved gating glutamate residues is typical for family members that function as channels.
Subcellular location: Membrane, Multi-pass membrane protein.
Tissue specificity: Predominantly expressed in skeletal muscles. Also known as: Skeletal muscle Chloride channel protein 1, ClC-1, CLCN1, CLC1.
Application Notes A dilution of 1 : 300 to 1 : 2000 is recommended.
The optimal dilution should be determined by the end user.
Not yet tested in other applications.
Restrictions For Research Use only
Format Lyophilized
Reconstitution Reconstitute in 100 µL of sterile water. Centrifuge to remove any insoluble material.
Handling Advice Avoid freeze and thaw cycles.
Storage 4 °C/-20 °C
Storage Comment Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.
Expiry Date 12 months
Background publications Sjöblom, Jones, Wood, Parsons, Lin, Barber, Mandelker, Leary, Ptak, Silliman, Szabo, Buckhaults, Farrell, Meeh, Markowitz, Willis, Dawson, Willson, Gazdar, Hartigan, Wu, Liu, Parmigiani, Park et al.: "The consensus coding sequences of human breast and colorectal cancers. ..." in: Science (New York, N.Y.), Vol. 314, Issue 5797, pp. 268-74, 2006 (PubMed).

Lehmann-Horn, Mailaender, Heine, George: "Myotonia levior is a chloride channel disorder." in: Human molecular genetics, Vol. 4, Issue 8, pp. 1397-402, 1995 (PubMed).

Lorenz, Meyer-Kleine, Steinmeyer, Koch, Jentsch: "Genomic organization of the human muscle chloride channel CIC-1 and analysis of novel mutations leading to Becker-type myotonia." in: Human molecular genetics, Vol. 3, Issue 6, pp. 941-6, 1994 (PubMed).

Steinmeyer, Lorenz, Pusch, Koch, Jentsch: "Multimeric structure of ClC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen)." in: The EMBO journal, Vol. 13, Issue 4, pp. 737-43, 1994 (PubMed).

Koch, Steinmeyer, Lorenz, Ricker, Wolf, Otto, Zoll, Lehmann-Horn, Grzeschik, Jentsch: "The skeletal muscle chloride channel in dominant and recessive human myotonia." in: Science (New York, N.Y.), Vol. 257, Issue 5071, pp. 797-800, 1992 (PubMed).