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hCLC1-G972 Blocking Peptide

Synthetic peptide

     
  • SPECIFICATION
  • CITATIONS
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Product Information
Primary Accession P35523
Other Accession P35524, Q64347
Additional Information
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.
StorageMaintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.
PrecautionsThis product is for research use only. Not for use in diagnostic or therapeutic procedures.
Protein Information
Name CLCN1 (HGNC:2019)
Synonyms CLC1
Function Voltage-gated chloride channel (PubMed:8112288, PubMed:9122265, PubMed:12456816). Plays an important role in membrane repolarization in skeletal muscle cells after muscle contraction. The CLC channel family contains both chloride channels and proton-coupled anion transporters that exchange chloride or another anion for protons (Probable). The absence of conserved gating glutamate residues is typical for family members that function as channels (Probable).
Cellular Location Cell membrane; Multi-pass membrane protein
Tissue Location Predominantly expressed in skeletal muscles.
Research Areas
Citations (0)

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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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$ 277.78
Cat# BP6329c
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Availability: 2 weeks
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