Please use this identifier to cite or link to this item: http://hdl.handle.net/2440/24087
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Type: Journal article
Title: ClC-1 chloride channel: Matching its properties to a role in skeletal muscle
Author: Aromataris, E.
Rychkov, G.
Citation: Clinical and Experimental Pharmacology and Physiology, 2006; 33(11):1118-1123
Publisher: Blackwell Publishing Asia
Issue Date: 2006
ISSN: 0305-1870
1440-1681
Statement of
Responsibility: 
Edoardo C Aromataris and Grigori Y Rychkov
Abstract: 1. ClC-1 is a Cl- channel in mammalian skeletal muscle that plays an important role in membrane repolarization following muscular contraction. Reduction of ClC-1 conductance results in myotonia, a state characterized by muscle hyperexcitability. 2. As is the case for other members of the ClC family, ClC-1 exists as a dimer that forms a double-barrelled channel. Each barrel, or pore, of ClC-1 is gated by its own gate ('fast' or 'single pore' gate), whereas both pores are gated simultaneously by another mechanism ('slow' or 'common' gate). 3. Comparison of the biophysical and pharmacological properties of heterologously expressed ClC-1 with the properties of the Cl- conductance measured in skeletal muscle strongly suggests that ClC-1 is the major Cl- channel responsible for muscle repolarization. However, not all results obtained in experiments on whole muscle or muscle fibres support this notion. 4. In the present review we attempt to bring together the current knowledge of ClC-1 with the physiology of skeletal muscle.
Keywords: Cl- conductance; ClC-1; gating; muscle excitability; myotonia congenita; skeletal muscle
Description: The definitive version is available at www.blackwell-synergy.com
RMID: 0020061506
DOI: 10.1111/j.1440-1681.2006.04502.x
Appears in Collections:Molecular and Biomedical Science publications

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