ArticlePflugers Archiv : European journal of physiology2010
ER-localized bestrophin 1 activates Ca2+-dependent ion channels TMEM16A and SK4 possibly by acting as a counterion channel.
Article in Pflugers Archiv : European journal of physiology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers.
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Who cites it
43 citing papers in PubMed, 86 citations in OpenAlex.
- TMEM16E-mediated macropinocytosis promotes cell survival under acidic stress.Cellular and molecular life sciences : CMLS · 2026Article
- CaActa physiologica (Oxford, England) · 2026Review
- Review of the clinical electrooculogram - Part 1: Mechanism of the Light-Rise.Documenta ophthalmologica. Advances in ophthalmology · 2026Review
- TMEM16A Contributes to Calcium-Activated Chloride Currents and Membrane Potential Changes in the Mouse Oocyte.Journal of cellular physiology · 2026Article
- Unilateral best vitelliform macular dystrophy- a case series.Documenta ophthalmologica. Advances in ophthalmology · 2025Article
- Identity revealed for a long-sought ER anion channel.Cell research · 2023Article
- Chloride Channels and Transporters: Roles beyond Classical Cellular Homeostatic pH or Ion Balance in Cancers.Cancers · 2022Review
- Is white the right light for the clinical electrooculogram?Documenta ophthalmologica. Advances in ophthalmology · 2021Article
- Evaluating BEST1 mutations in pluripotent stem cell-derived retinal pigment epithelial cells.Methods in enzymology · 2021Article
- Review
- Article
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- Ion channel signaling influences cellular proliferation and phagocyte activity during axolotl tail regeneration.Mechanisms of development · 2017Article
- Bestrophin 1 and retinal disease.Progress in retinal and eye research · 2017Review
- Review
- Restoration of mutant bestrophin-1 expression, localisation and function in a polarised epithelial cell model.Disease models & mechanisms · 2016Article
- Store-operated Ca2+ entry regulates Ca2+-activated chloride channels and eccrine sweat gland function.The Journal of clinical investigation · 2016Article
- BESTROPHIN1 mutations cause defective chloride conductance in patient stem cell-derived RPE.Human molecular genetics · 2016Article
- A C-terminally truncated mouse Best3 splice variant targets and alters the ion balance in lysosome-endosome hybrids and the endoplasmic reticulum.Scientific reports · 2016Article
- Modulating Ca²⁺ signals: a common theme for TMEM16, Ist2, and TMC.Pflugers Archiv : European journal of physiology · 2016Review
Corrections and comments
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Bestrophins form Ca(2+)-activated Cl(-) channels and regulate intracellular Ca(2+) signaling. We demonstrate that bestrophin 1 is localized in the endoplasmic reticulum (ER), where it interacts with stromal interacting molecule 1, the ER-Ca(2+) sensor. Intracellular Ca(2+) transients elicited by stimulation of purinergic P2Y(2) receptors in HEK293 cells were augmented by hBest1. The p21-activated protein kinase Pak2 was found to phosphorylate hBest1, thereby enhancing Ca(2+) signaling and activation of Ca(2+)-dependent Cl(-) (TMEM16A) and K(+) (SK4) channels. Lack of bestrophin 1 expression in respiratory epithelial cells of mBest1 knockout mice caused expansion of ER cisterns and induced Ca(2+) deposits. hBest1 is, therefore, important for Ca(2+) handling of the ER store and may resemble the long-suspected counterion channel to balance transient membrane potentials occurring through inositol triphosphate (IP(3))-induced Ca(2+) release and store refill. Thus, bestrophin 1 regulates compartmentalized Ca(2+) signaling that plays an essential role in Best macular dystrophy, inflammatory diseases such as cystic fibrosis, as well as proliferation.
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Registered trials
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