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Molecular mechanisms of gating in the calcium-activated chloride channel bestrophin.
Miller AN, Vaisey G, Long SB. Miller AN, et al. Elife. 2019 Jan 10;8:e43231. doi: 10.7554/eLife.43231. Elife. 2019. PMID: 30628889 Free PMC article.
Bestrophin (BEST1-4) ligand-gated chloride (Cl(-)) channels are activated by calcium (Ca(2+)). Mutation of BEST1 causes retinal disease. Partly because bestrophin channels have no sequence or structural similarity to other i
Bestrophin (BEST1-4) ligand-gated chloride (Cl(-)) channels are activated by calcium (Ca(2+)). Mut
Structure and Function of the Bestrophin family of calcium-activated chloride channels.
Owji AP, Kittredge A, Zhang Y, Yang T. Owji AP, et al. Channels (Austin). 2021 Dec;15(1):604-623. doi: 10.1080/19336950.2021.1981625. Channels (Austin). 2021. PMID: 34612806 Free PMC article. Review.
Bestrophins are a family of calcium-activated chloride channels (CaCCs) with relevance to human physiology and a myriad of eye diseases termed "bestrophinopathies". Since the identification of bestrophins as CaCCs nearly two decades ago,
Bestrophins are a family of calcium-activated chloride channels (CaCCs) with relevance to human physiolog
Mutation-Dependent Pathomechanisms Determine the Phenotype in the Bestrophinopathies.
Nachtigal AL, Milenkovic A, Brandl C, Schulz HL, Duerr LMJ, Lang GE, Reiff C, Herrmann P, Kellner U, Weber BHF. Nachtigal AL, et al. Int J Mol Sci. 2020 Feb 26;21(5):1597. doi: 10.3390/ijms21051597. Int J Mol Sci. 2020. PMID: 32111077 Free PMC article.
Both BD and ARB hiPSC-RPEs display a strong reduction of BEST1-mediated anion transport function compared to control, while ADVIRC mutations trigger an increased anion permeability suggesting a stabilized open state condition of channel gating. Furthermore, BD and A …
Both BD and ARB hiPSC-RPEs display a strong reduction of BEST1-mediated anion transport function compared to control, while ADVIRC mutations …
Structures and gating mechanisms of human bestrophin anion channels.
Owji AP, Wang J, Kittredge A, Clark Z, Zhang Y, Hendrickson WA, Yang T. Owji AP, et al. Nat Commun. 2022 Jul 4;13(1):3836. doi: 10.1038/s41467-022-31437-7. Nat Commun. 2022. PMID: 35789156 Free PMC article.
Bestrophin-1 (Best1) and bestrophin-2 (Best2) are two members of the bestrophin family of calcium (Ca(2+))-activated chloride (Cl(-)) channels with critical involvement in ocular physiology and direct pathological relevance. ...Com
Bestrophin-1 (Best1) and bestrophin-2 (Best2) are two members of the bestrophin family of calcium (Ca(2+))-ac
Transport and function of chloride in vascular smooth muscles.
Matchkov VV, Secher Dam V, Bødtkjer DM, Aalkjær C. Matchkov VV, et al. J Vasc Res. 2013;50(1):69-87. doi: 10.1159/000345242. Epub 2012 Nov 17. J Vasc Res. 2013. PMID: 23172353 Review.
The cystic fibrosis transmembrane conductance regulator is also demonstrated in VSMCs. The molecular identity of the major anion conductance in VSMCs - a Ca(2+)-activated Cl(-) current - is uncertain. Several candidates have been suggested with bestrophin and …
The cystic fibrosis transmembrane conductance regulator is also demonstrated in VSMCs. The molecular identity of the major anion cond …
Role of Cl- channels in primary brain tumour.
Saberbaghi T, Wong R, Rutka JT, Wang GL, Feng ZP, Sun HS. Saberbaghi T, et al. Cell Calcium. 2019 Jul;81:1-11. doi: 10.1016/j.ceca.2019.05.004. Epub 2019 May 14. Cell Calcium. 2019. PMID: 31129471 Review.
Glioma is the predominant malignant primary brain tumour, accounting for 80% of all cases. Voltage-gated Cl(-) channel family (ClC) proteins and Cl(-) intracellular channel (CLIC) proteins are drastically overexpressed in glioma, and are associated with enhan …
Glioma is the predominant malignant primary brain tumour, accounting for 80% of all cases. Voltage-gated Cl(-) channel family …
Structure and insights into the function of a Ca(2+)-activated Cl(-) channel.
Kane Dickson V, Pedi L, Long SB. Kane Dickson V, et al. Nature. 2014 Dec 11;516(7530):213-8. doi: 10.1038/nature13913. Epub 2014 Oct 22. Nature. 2014. PMID: 25337878 Free PMC article.
Bestrophin calcium-activated chloride channels (CaCCs) regulate the flow of chloride and other monovalent anions across cellular membranes in response to intracellular calcium (Ca(2+)) levels. ...Conformational changes observed nea
Bestrophin calcium-activated chloride channels (CaCCs) regulate the flow of chloride and other mon
Cryo-EM structures of thylakoid-located voltage-dependent chloride channel VCCN1.
Hagino T, Kato T, Kasuya G, Kobayashi K, Kusakizako T, Hamamoto S, Sobajima T, Fujiwara Y, Yamashita K, Kawasaki H, Maturana AD, Nishizawa T, Nureki O. Hagino T, et al. Nat Commun. 2022 May 6;13(1):2505. doi: 10.1038/s41467-022-30292-w. Nat Commun. 2022. PMID: 35523970 Free PMC article.
Here, we present the cryo-EM structures of Malus domestica VCCN1 (MdVCCN1) in nanodiscs and detergent at 2.7 A and 3.0 A resolutions, respectively, and the structure-based electrophysiological analyses. VCCN1 structurally resembles its animal homolog, bestrophin, a Ca(2+)- …
Here, we present the cryo-EM structures of Malus domestica VCCN1 (MdVCCN1) in nanodiscs and detergent at 2.7 A and 3.0 A resolutions, respec …
Calcium-activated chloride currents in olfactory sensory neurons from mice lacking bestrophin-2.
Pifferi S, Dibattista M, Sagheddu C, Boccaccio A, Al Qteishat A, Ghirardi F, Tirindelli R, Menini A. Pifferi S, et al. J Physiol. 2009 Sep 1;587(Pt 17):4265-79. doi: 10.1113/jphysiol.2009.176131. Epub 2009 Jul 21. J Physiol. 2009. PMID: 19622610 Free PMC article.
Ca(2+) activates a Cl(-) current that produces an efflux of Cl(-) ions and amplifies the depolarization. The molecular identity of Ca(2+)-activated Cl(-) channels is still elusive, although some bestrophins have been shown to function as Ca(2+)-acti
Ca(2+) activates a Cl(-) current that produces an efflux of Cl(-) ions and amplifies the depolarization. The molecular identity of Ca …
Regulation of bestrophin Cl channels by calcium: role of the C terminus.
Xiao Q, Prussia A, Yu K, Cui YY, Hartzell HC. Xiao Q, et al. J Gen Physiol. 2008 Dec;132(6):681-92. doi: 10.1085/jgp.200810056. J Gen Physiol. 2008. PMID: 19029375 Free PMC article.
Human bestrophin-1 (hBest1), which is genetically linked to several kinds of retinopathy and macular degeneration in both humans and dogs, is the founding member of a family of Cl(-) ion channels that are activated by intracellular Ca(2+). ...The cluster of f …
Human bestrophin-1 (hBest1), which is genetically linked to several kinds of retinopathy and macular degeneration in both humans and …
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