The best disease-linked Cl- channel hBest1 regulates Ca V 1 (L-type) Ca2+ channels via src-homology-binding domains.
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 membersChloride channels as drug targets.Dysregulation of human bestrophin-1 by ceramide-induced dephosphorylationFunctional roles of bestrophins in ocular epitheliaHuman disease-causing mutations disrupt an N-C-terminal interaction and channel function of bestrophin 1Interaction 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 channelsRegulation of bestrophins by Ca2+: a theoretical and experimental study.The beta subunit of voltage-gated Ca2+ channels interacts with and regulates the activity of a novel isoform of Pax6.Enhanced inflow and outflow rates despite lower IOP in bestrophin-2-deficient mice.Chloride channels: often enigmatic, rarely predictable.The Cavβ1a subunit regulates gene expression and suppresses myogenin in muscle progenitor cells.Bestrophins and retinopathies.Role of the Ca2+ -activated Cl- channels bestrophin and anoctamin in epithelial cells.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 neuronsBestrophin 1 and retinal disease.Autosomal Recessive Bestrophinopathy Is Not Associated With the Loss of Bestrophin-1 Anion Channel Function in a Patient With a Novel BEST1 MutationThree-dimensional distribution of the vitelliform lesion, photoreceptors, and retinal pigment epithelium in the macula of patients with best vitelliform macular dystrophy.Rescue of volume-regulated anion current by bestrophin mutants with altered charge selectivity.Phenotypic variability due to a novel Glu292Lys variation in exon 8 of the BEST1 gene causing best macular dystrophy.Calcium binding protein-mediated regulation of voltage-gated calcium channels linked to human diseases.Calcium-activated chloride channels in the corpus cavernosum: recent developments and future of a key cellular component of the erectile process.Bestrophin 1--Phenotypes and Functional Aspects in Bestrophinopathies.Negative News: Cl- and HCO3- in the Vascular Wall.Characterization of the effects of Cl⁻ channel modulators on TMEM16A and bestrophin-1 Ca²⁺ activated Cl⁻ channels.Suppression of Ca2+ signaling in a mouse model of Best disease.Bestrophin-1 influences transepithelial electrical properties and Ca2+ signaling in human retinal pigment epithelium.Disease-associated missense mutations in bestrophin-1 affect cellular trafficking and anion conductance.Bestrophin 2 is expressed in human non-pigmented ciliary epithelium but not retinal pigment epithelium.Plasticity of L-type Ca2+ channels after cocaine withdrawal.TMEM16A knockdown abrogates two different Ca(2+)-activated Cl (-) currents and contractility of smooth muscle in rat mesenteric small arteriesDisease-causing mutations associated with four bestrophinopathies exhibit disparate effects on the localization, but not the oligomerization, of Bestrophin-1.Best1 is a gene regulated by nerve injury and required for Ca2+-activated Cl- current expression in axotomized sensory neurons.Patient-specific mutations impair BESTROPHIN1's essential role in mediating Ca2+-dependent Cl- currents in human RPE.Bestrophin-1 encodes for the Ca2+-activated anion channel in hippocampal astrocytes.Differential effects of Best disease causing missense mutations on bestrophin-1 trafficking.
P2860
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P2860
The best disease-linked Cl- channel hBest1 regulates Ca V 1 (L-type) Ca2+ channels via src-homology-binding domains.
description
2008 nî lūn-bûn
@nan
2008 թուականի Մայիսին հրատարակուած գիտական յօդուած
@hyw
2008 թվականի մայիսին 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
The best disease-linked Cl- ch ...... src-homology-binding domains.
@ast
The best disease-linked Cl- ch ...... src-homology-binding domains.
@en
type
label
The best disease-linked Cl- ch ...... src-homology-binding domains.
@ast
The best disease-linked Cl- ch ...... src-homology-binding domains.
@en
prefLabel
The best disease-linked Cl- ch ...... src-homology-binding domains.
@ast
The best disease-linked Cl- ch ...... src-homology-binding domains.
@en
P2093
P2860
P1476
The best disease-linked Cl- ch ...... src-homology-binding domains.
@en
P2093
Guiying Cui
H Criss Hartzell
Qinghuan Xiao
P2860
P304
P356
10.1523/JNEUROSCI.0065-08.2008
P407
P50
P577
2008-05-01T00:00:00Z