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Int. J. Mol. Sci. 2014, 15(1), 218-249; doi:10.3390/ijms15010218

Chloride Channelopathies of ClC-2

1 Department of Ophthalmology, the Second Hospital of Jilin University, Jilin University, Changchun 130041, Jilin, China 2 Department of Ophthalmology, the China-Japan Union Hospital, Jilin University, Changchun 130033, Jilin, China 3 Department of Colon and Anal Surgery, the First Hospital of Jilin University, Jilin University, Changchun 130021, Jilin, China 4 Department of Ophthalmology, the First Affiliated Hospital of Qiqihar Medical College, Qiqihar Medical University, Qiqihar 161006, Heilongjiang, China These authors contributed equally to this work.
* Author to whom correspondence should be addressed.
Received: 26 September 2013 / Revised: 14 November 2013 / Accepted: 16 December 2013 / Published: 27 December 2013
(This article belongs to the Section Biochemistry, Molecular Biology and Biophysics)
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Chloride channels (ClCs) have gained worldwide interest because of their molecular diversity, widespread distribution in mammalian tissues and organs, and their link to various human diseases. Nine different ClCs have been molecularly identified and functionally characterized in mammals. ClC-2 is one of nine mammalian members of the ClC family. It possesses unique biophysical characteristics, pharmacological properties, and molecular features that distinguish it from other ClC family members. ClC-2 has wide organ/tissue distribution and is ubiquitously expressed. Published studies consistently point to a high degree of conservation of ClC-2 function and regulation across various species from nematodes to humans over vast evolutionary time spans. ClC-2 has been intensively and extensively studied over the past two decades, leading to the accumulation of a plethora of information to advance our understanding of its pathophysiological functions; however, many controversies still exist. It is necessary to analyze the research findings, and integrate different views to have a better understanding of ClC-2. This review focuses on ClC-2 only, providing an analytical overview of the available literature. Nearly every aspect of ClC-2 is discussed in the review: molecular features, biophysical characteristics, pharmacological properties, cellular function, regulation of expression and function, and channelopathies.
Keywords: ClCs; ClC-2; ClC-2 channels; ClC-2 chloride channelopathies ClCs; ClC-2; ClC-2 channels; ClC-2 chloride channelopathies
This is an open access article distributed under the Creative Commons Attribution License (CC BY) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Bi, M.M.; Hong, S.; Zhou, H.Y.; Wang, H.W.; Wang, L.N.; Zheng, Y.J. Chloride Channelopathies of ClC-2. Int. J. Mol. Sci. 2014, 15, 218-249.

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