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Discovery of CLC transport proteins: cloning, structure, function and pathophysiology

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Item Type:Review
Title:Discovery of CLC transport proteins: cloning, structure, function and pathophysiology
Creators Name:Jentsch, T.J.
Abstract:After a personal description of the convoluted path leading 25 years ago to the molecular identification of the Torpedo Cl(-) channel ClC-0 and the discovery of the CLC gene family, I succinctly describe general structural and functional features of these ion transporters before giving a short overview of mammalian CLCs. These can be categorized into plasma membrane Cl(-) channels and vesicular Cl(-) /H(+) -exchangers. They are involved in the regulation of membrane excitability, transepithelial transport, extracellular ion homeostasis, endocytosis, and lysosomal function. Diseases caused by CLC dysfunction include myotonia, neurodegeneration, deafness, blindness, leukodystrophy, male infertility, renal salt loss, kidney stones and osteopetrosis, revealing a surprisingly broad spectrum of biological roles for chloride transport that was unsuspected when I set out to clone the first voltage-gated chloride channel.
Keywords:Biological Transport, Carrier Proteins, Cell Membrane, Chloride Channels, Chlorides, Ion Transport, Molecular Cloning, Animals
Source:Journal of Physiology
ISSN:0022-3751
Publisher:Wiley-Blackwell (U.K.)
Volume:593
Number:18
Page Range:4091-4109
Date:15 September 2015
Official Publication:https://doi.org/10.1113/JP270043
PubMed:View item in PubMed

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