The DOI matches its Crossref record; 12 of the 12 references it cites were found in a registry or an index.
CLC Chloride Channels and Transporters: Structure, Function, Physiology, and Disease
Open accessAbstract
CLC anion transporters are found in all phyla and form a gene family of eight members in mammals. Two CLC proteins, each of which completely contains an ion translocation parthway, assemble to homo- or heteromeric dimers that sometimes require accessory β-subunits for function. CLC proteins come in two flavors: anion channels and anion/proton exchangers. Structures of these two CLC protein classes are surprisingly similar. Extensive structure-function analysis identified residues involved in ion permeation, anion-proton coupling and gating and led to attractive biophysical models. In mammals, ClC-1, -2, -Ka/-Kb are plasma membrane Cl−channels, whereas ClC-3 through ClC-7 are 2Cl−/H+-exchangers in endolysosomal membranes. Biological roles of CLCs were mostly studied in mammals, but also in plants and model organisms like yeast and Caenorhabditis elegans. CLC Cl−channels have roles in the control of electrical excitability, extra- and intracellular ion homeostasis, and transepithelial transport, whereas anion/proton exchangers influence vesicular ion composition and impinge on endocytosis and lysosomal function. The surprisingly diverse roles of CLCs are highlighted by human and mouse disorders elicited by mutations in their genes. These pathologies include neurodegeneration, leukodystrophy, mental retardation, deafness, blindness, myotonia, hyperaldosteronism, renal salt loss, proteinuria, kidney stones, male infertility, and osteopetrosis. In this review, emphasis is laid on biophysical structure-function analysis and on the cell biological and organismal roles of mammalian CLCs and their role in disease.
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Molecular Basis of CLC Antiporter Inhibition by Fluoride
Chiariello, M. G., Bolnykh, V., Ippoliti, E., Meloni, S., Olsen, J. M. H., Beck, T., Rothlisberger, U., Fahlke, C., & Carloni, P. (2020) · Journal of the American Chemical Society · doi:10.1021/jacs.9b13588 · Cited by 31
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The identification of XPR1 as a voltage- and phosphate-activated phosphate-permeable ion channel
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Metadata
- Authors
- Jentsch, T. J., & Pusch, M.
- Venue
- Physiological Reviews
- Publisher
- American Physiological Society
- Published
- 2018-07-01
- Volume
- 98
- Issue
- 3
- Pages
- 1493-1590
- ISSN
- 0031-9333 (ISSN-L)
- 1522-1210
- OpenAlex
- W2805997389
- Cited by
- 448
- References
- 12
- FWCI
- 3.41
- Type
- journal article
- Language
- en
- Open access
- bronze
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