|Other Accession||P35524, Q64347|
|Other Names||Chloride channel protein 1, ClC-1, Chloride channel protein, skeletal muscle, CLCN1, CLC1|
|Target/Specificity||The synthetic peptide sequence is selected from aa 972~988 of HUMAN CLCN1|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Voltage-gated chloride channel. Chloride channels have several functions including the regulation of cell volume; membrane potential stabilization, signal transduction and transepithelial transport.|
|Cellular Location||Membrane; Multi-pass membrane protein.|
|Tissue Location||Predominantly expressed in skeletal muscles.|
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Provided below are standard protocols that you may find useful for product applications.
The CLCN family of voltage-dependent chloride channel genes comprises nine members (CLCN1-7, Ka and Kb) which demonstrate quite diverse functional characteristics while sharing significant sequence homology. The protein encoded by this gene regulates the electric excitability of the skeletal muscle membrane. Mutations in this gene cause two forms of inherited human muscle disorders: recessive generalized myotonia congenita (Becker) and dominant myotonia (Thomsen).
Jou, S.B., et al., J. Neurol. 251(6):666-670 (2004).
Hebeisen, S., et al., J. Biol. Chem. 279(13):13140-13147 (2004).
Letizia, C., et al., Calcif. Tissue Int. 74(1):42-46 (2004).
Estevez, R., et al., Neuron 38(1):47-59 (2003).
Pusch, M., Hum. Mutat. 19(4):423-434 (2002).
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