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hCLC1-G972 Blocking Peptide
Synthetic peptide
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
| Primary Accession | P35523 |
|---|---|
| Other Accession | P35524, Q64347 |
| Gene ID | 1180 |
|---|---|
| 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 | Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed. |
| 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. |
| Name | CLCN1 {ECO:0000303|PubMed:8533761, ECO:0000312|HGNC:HGNC:2019} |
|---|---|
| Function | Voltage-gated chloride channel involved in skeletal muscle excitability. Generates most of the plasma membrane chloride conductance in skeletal muscle fibers, stabilizes the resting membrane potential and contributes to the repolarization phase during action potential firing (PubMed:12456816, PubMed:16027167, PubMed:22521272, PubMed:22641783, PubMed:26007199, PubMed:26502825, PubMed:26510092, PubMed:7951242, PubMed:8112288, PubMed:8130334, PubMed:9122265, PubMed:9565403, PubMed:9736777). Forms a homodimeric channel where each subunit has its own ion conduction pathway. Conducts double-barreled currents controlled by two types of gates, two fast glutamate gates that control each subunit independently and a slow common gate that opens and shuts off both subunits simultaneously. Has a significant open probability at muscle resting potential and is further activated upon membrane depolarization (PubMed:10051520, PubMed:10962018, PubMed:29809153, PubMed:31022181). Permeable to small monovalent anions with ion selectivity for chloride > thiocyanate > bromide > nitrate > iodide (PubMed:9122265, PubMed:9565403). |
| Cellular Location | Cell membrane; Multi-pass membrane protein Cell membrane, sarcolemma {ECO:0000250|UniProtKB:Q64347}; Multi-pass membrane protein. Cell membrane, sarcolemma, T-tubule {ECO:0000250|UniProtKB:Q64347}; 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.
Background
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).
References
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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