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.
Overview
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Citations
Cites
Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA
Riordan, J. R., Rommens, J. M., Kerem, B.-S., Alon, N., Rozmahel, R., Grzelczak, Z., Zielenski, J., Lok, S., Plavsic, N., Chou, J.-L., et al. (1989) · Science · doi:10.1126/science.2475911 · Cited by 7,418
A Gene Network Regulating Lysosomal Biogenesis and Function
Sardiello, M., Palmieri, M., di Ronza, A., Medina, D. L., Valenza, M., Gennarino, V. A., Di Malta, C., Donaudy, F., Embrione, V., Polishchuk, R. S., et al. (2009) · Science · doi:10.1126/science.1174447 · Cited by 2,543
Molecular anatomy of a trafficking organelle
Takamori, S., Holt, M., Stenius, K., Lemke, E. A., Grønborg, M., Riedel, D., Urlaub, H., Schenck, S., Brügger, B., Ringler, P., et al. (2006) · Cell · doi:10.1016/j.cell.2006.10.030 · Cited by 2,373
Structure and mechanism of carbonic anhydrase
Lindskog, S. (1997) · Pharmacology & Therapeutics · doi:10.1016/s0163-7258(96)00198-2 · Cited by 1,022
Scott, J. W., Hawley, S. A., Green, K. A., Anis, M., Stewart, G., Scullion, G. A., Norman, D. G., & Hardie, D. G. (2004) · Journal of Clinical Investigation · doi:10.1172/jci19874 · Cited by 754
SWELL1, a Plasma Membrane Protein, Is an Essential Component of Volume-Regulated Anion Channel
Qiu, Z., Dubin, A. E., Mathur, J., Tu, B., Reddy, K., Miraglia, L. J., Reinhardt, J., Orth, A. P., & Patapoutian, A. (2014) · Cell · doi:10.1016/j.cell.2014.03.024 · Cited by 571
Control of aldosterone secretion: a model for convergence in cellular signaling pathways
SPÄT, A., & HUNYADY, L. (2004) · Physiological Reviews · doi:10.1152/physrev.00030.2003 · Cited by 494
The Cl-/H+ antiporter ClC-7 is the primary chloride permeation pathway in lysosomes
Graves, A. R., Curran, P. K., Smith, C. L., & Mindell, J. A. (2008) · Nature · doi:10.1038/nature06907 · Cited by 399
Priming of insulin granules for exocytosis by granular Cl(-) uptake and acidification
Barg, S., Huang, P., Eliasson, L., Nelson, D. J., Obermüller, S., Rorsman, P., Thévenod, F., & Renström, E. (2001) · Journal of Cell Science · doi:10.1242/jcs.114.11.2145 · Cited by 194
Receptors, Ion Channels, and Signaling Mechanisms Underlying Microglial Dynamics
Madry, C., & Attwell, D. (2015) · Journal of Biological Chemistry · doi:10.1074/jbc.r115.637157 · Cited by 109
Li, D.-Q., Jing, X., Salehi, A., Collins, S. C., Hoppa, M. B., Rosengren, A. H., Zhang, E., Lundquist, I., Olofsson, C. S., Mörgelin, M., et al. (2009) · Cell Metabolism · doi:10.1016/j.cmet.2009.08.011 · Cited by 50
Guzman, R. E., Alekov, A. K., Filippov, M., Hegermann, J., & Fahlke, C. (2014) · Frontiers in Cellular Neuroscience · doi:10.3389/fncel.2014.00143 · Cited by 38
Cited by
Developmental and epileptic encephalopathies: from genetic heterogeneity to phenotypic continuum
Guerrini, R., Conti, V., Mantegazza, M., Balestrini, S., Galanopoulou, A. S., & Benfenati, F. (2023) · Physiological Reviews · doi:10.1152/physrev.00063.2021 · Cited by 196
Membrane exporters of fluoride ion
McIlwain, B. C., Ruprecht, M. T., & Stockbridge, R. B. (2021) · Annual Review of Biochemistry · doi:10.1146/annurev-biochem-071520-112507 · Cited by 69
Maggi, L., Bonanno, S., Altamura, C., & Desaphy, J.-F. (2021) · Cells · doi:10.3390/cells10061521 · Cited by 51
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
Lanciotti, A., Brignone, M. S., Macioce, P., Visentin, S., & Ambrosini, E. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms23010274 · Cited by 14
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
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
Data sources
Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
