ClC-1 CHLORIDE CHANNEL: MATCHING ITS PROPERTIES TO A ROLE IN SKELETAL MUSCLE

Authors: Aromataris, Edoardo C; Rychkov, Grigori Y

Source: Clinical and Experimental Pharmacology and Physiology, Volume 33, Number 11, November 2006 , pp. 1118-1123(6)

Publisher: Wiley-Blackwell

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Abstract:

SUMMARY

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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.

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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).

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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.

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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

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1440-1681.2006.04502.x

Publication date: 2006-11-01