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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 |
| 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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