Molecular physiology of bestrophins: multifunctional membrane proteins linked to best disease and other retinopathies
about
Missense mutations in a retinal pigment epithelium protein, bestrophin-1, cause retinitis pigmentosaSOX9, through interaction with microphthalmia-associated transcription factor (MITF) and OTX2, regulates BEST1 expression in the retinal pigment epitheliumBEST1 expression in the retinal pigment epithelium is modulated by OTX family membersAnoctamin/TMEM16 family members are Ca2+-activated Cl- channelsDysregulation of human bestrophin-1 by ceramide-induced dephosphorylationRegulation of bestrophin Cl channels by calcium: role of the C terminusFunctional roles of bestrophins in ocular epitheliaNew Insights on Astrocyte Ion Channels: Critical for Homeostasis and Neuron-Glia SignalingA C-terminally truncated mouse Best3 splice variant targets and alters the ion balance in lysosome-endosome hybrids and the endoplasmic reticulum.Human disease-causing mutations disrupt an N-C-terminal interaction and channel function of bestrophin 1Ion transporting proteins of human bronchial epitheliumBestrophin Cl- channels are highly permeable to HCO3-Interaction of bestrophin-1 and Ca2+ channel β-subunits: identification of new binding domains on the bestrophin-1 C-terminusThe ß subunit of voltage-gated Ca2+ channelsEffects of alternative splicing on the function of bestrophin-1 calcium-activated chloride channelsThe best disease-linked Cl- channel hBest1 regulates Ca V 1 (L-type) Ca2+ channels via src-homology-binding domains.The Concise Guide to PHARMACOLOGY 2013/14: ion channelsStoichiometry and specific assembly of Best ion channelsBestrophin 1 is indispensable for volume regulation in human retinal pigment epithelium cells.Drosophila bestrophin-1 chloride current is dually regulated by calcium and cell volume.Lineage-specific expression of bestrophin-2 and bestrophin-4 in human intestinal epithelial cellsRegulation of bestrophins by Ca2+: a theoretical and experimental study.Enhanced inflow and outflow rates despite lower IOP in bestrophin-2-deficient mice.Chloride channels: often enigmatic, rarely predictable.Tmem16A encodes the Ca2+-activated Cl- channel in mouse submandibular salivary gland acinar cellsBestrophin-2 mediates bicarbonate transport by goblet cells in mouse colonDistribution and Function of the Bestrophin-1 (Best1) Channel in the BrainPhotoreceptor degeneration, azoospermia, leukoencephalopathy, and abnormal RPE cell function in mice expressing an early stop mutation in CLCN2.The retinal pigment epithelium in health and diseaseExpression of novel opsins and intrinsic light responses in the mammalian retinal ganglion cell line RGC-5. Presence of OPN5 in the rat retinaChloride channels of intracellular membranesBestrophins and retinopathies.Role of the Ca2+ -activated Cl- channels bestrophin and anoctamin in epithelial cells.International Union of Basic and Clinical Pharmacology. LXXXV: calcium-activated chloride channels.What do we know about gliotransmitter release from astrocytes?Physiology of epithelial chloride and fluid secretionAssessment of canine BEST1 variations identifies new mutations and establishes an independent bestrophinopathy model (cmr3).Anion-sensitive fluorophore identifies the Drosophila swell-activated chloride channel in a genome-wide RNA interference screen.Bestrophin-encoded Ca²⁺-activated Cl⁻ channels underlie a current with properties similar to the native current in the moth Spodoptera littoralis olfactory receptor neuronsPhenotypic variability in a French family with a novel mutation in the BEST1 gene causing multifocal best vitelliform macular dystrophy.
P2860
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P2860
Molecular physiology of bestrophins: multifunctional membrane proteins linked to best disease and other retinopathies
description
2008 nî lūn-bûn
@nan
2008 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
2008 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
name
Molecular physiology of bestro ...... isease and other retinopathies
@ast
Molecular physiology of bestro ...... isease and other retinopathies
@en
Molecular physiology of bestro ...... isease and other retinopathies
@nl
type
label
Molecular physiology of bestro ...... isease and other retinopathies
@ast
Molecular physiology of bestro ...... isease and other retinopathies
@en
Molecular physiology of bestro ...... isease and other retinopathies
@nl
prefLabel
Molecular physiology of bestro ...... isease and other retinopathies
@ast
Molecular physiology of bestro ...... isease and other retinopathies
@en
Molecular physiology of bestro ...... isease and other retinopathies
@nl
P2093
P2860
P3181
P1476
Molecular physiology of bestro ...... isease and other retinopathies
@en
P2093
H Criss Hartzell
Li-Ting Chien
Qinghuan Xiao
Zhiqiang Qu
P2860
P304
P3181
P356
10.1152/PHYSREV.00022.2007
P407
P577
2008-04-01T00:00:00Z