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

Chloride Channelopathies of ClC-2

1,2,†email, 3,†email, 2email, 4email, 2email and 1,* email
Received: 26 September 2013; in revised form: 14 November 2013 / Accepted: 16 December 2013 / Published: 27 December 2013
(This article belongs to the Section Biochemistry, Molecular Biology and Biophysics)
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract: 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
PDF Full-text Download PDF Full-Text [632 KB, uploaded 19 June 2014 05:22 CEST]

Export to BibTeX |
EndNote


MDPI and ACS Style

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.

AMA Style

Bi MM, Hong S, Zhou HY, Wang HW, Wang LN, Zheng YJ. Chloride Channelopathies of ClC-2. International Journal of Molecular Sciences. 2014; 15(1):218-249.

Chicago/Turabian Style

Bi, Miao M.; Hong, Sen; Zhou, Hong Y.; Wang, Hong W.; Wang, Li N.; Zheng, Ya J. 2014. "Chloride Channelopathies of ClC-2." Int. J. Mol. Sci. 15, no. 1: 218-249.


Int. J. Mol. Sci. EISSN 1422-0067 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert