ArticleInvestigative ophthalmology & visual science2004
A model of best vitelliform macular dystrophy in rats.
Article in Investigative ophthalmology & visual science, 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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Who cites it
26 citing papers in PubMed, 56 citations in OpenAlex.
- Review of the clinical electrooculogram - Part 1: Mechanism of the Light-Rise.Documenta ophthalmologica. Advances in ophthalmology · 2026Review
- Bestrophin 1 and retinal disease.Progress in retinal and eye research · 2017Review
- Bestrophinopathy: An RPE-photoreceptor interface disease.Progress in retinal and eye research · 2017Review
- Bestrophin-1 influences transepithelial electrical properties and Ca2+ signaling in human retinal pigment epithelium.Molecular vision · 2015Article
- Stoichiometry and specific assembly of Best ion channels.Proceedings of the National Academy of Sciences of the United States of America · 2014Article
- Disease-causing mutations associated with four bestrophinopathies exhibit disparate effects on the localization, but not the oligomerization, of Bestrophin-1.Experimental eye research · 2014Article
- Differential effects of Best disease causing missense mutations on bestrophin-1 trafficking.Human molecular genetics · 2013Article
- Disease-causing mutations in BEST1 gene are associated with altered sorting of bestrophin-1 protein.International journal of molecular sciences · 2013Article
- Human photoreceptor outer segments shorten during light adaptation.Investigative ophthalmology & visual science · 2013Article
- Clinical evaluation of two consanguineous families with homozygous mutations in BEST1.Molecular vision · 2011Article
- Bestrophin 2 is expressed in human non-pigmented ciliary epithelium but not retinal pigment epithelium.Molecular vision · 2010Article
- Best1 is a gene regulated by nerve injury and required for Ca2+-activated Cl- current expression in axotomized sensory neurons.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2009Article
- Phenotypic variability due to a novel Glu292Lys variation in exon 8 of the BEST1 gene causing best macular dystrophy.Archives of ophthalmology (Chicago, Ill. : 1960) · 2009Article
- Detailed analysis of retinal function and morphology in a patient with autosomal recessive bestrophinopathy (ARB).Documenta ophthalmologica. Advances in ophthalmology · 2009Article
- Functional roles of bestrophins in ocular epithelia.Progress in retinal and eye research · 2009Review
- Rescue of volume-regulated anion current by bestrophin mutants with altered charge selectivity.The Journal of general physiology · 2008Article
- Bestrophin Cl- channels are highly permeable to HCO3-.American journal of physiology. Cell physiology · 2008Article
- The best disease-linked Cl- channel hBest1 regulates Ca V 1 (L-type) Ca2+ channels via src-homology-binding domains.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2008Article
- Bestrophin gene mutations cause canine multifocal retinopathy: a novel animal model for best disease.Investigative ophthalmology & visual science · 2007Article
- Voltage-dependent calcium channel CaV1.3 subunits regulate the light peak of the electroretinogram.Journal of neurophysiology · 2007Article
Corrections and comments
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Authors and funding
8 authors at 3 institutions in 2 countries.
Funding
Abstract
purposeThe VMD2 gene, mutated in Best macular dystrophy (BMD) encodes bestrophin, a 68-kDa basolateral plasma membrane protein expressed in retinal pigment epithelial (RPE) cells. BMD is characterized by a depressed light peak (LP) in the electro-oculogram. Bestrophin is thought to be the Cl channel that generates the LP. The goal was to generate an animal model of BMD and to determine the effects of bestrophin overexpression on the RPE-generated components of the ERG.
methodsBestrophin or bestrophin mutants (W93C or R218C) were overexpressed in the RPE of rats by injection of replication-defective adenovirus. Immunofluorescence microscopy and ERG recordings were used to study subsequent effects.
resultsBestrophin was confined to the basolateral plasma membrane of the RPE. Neither wild-type (wt) nor mutant bestrophin affected the a- or b-waves of the ERG. Wt bestrophin, however, increased the c-wave and fast oscillation (FO), but not the LP. In contrast, both mutants had little or no effect on the c-wave and FO, but did reduce LP amplitude. LP amplitudes across a range of stimuli were not altered by wt bestrophin, though the luminance response function was desensitized. LP response functions were unaffected by bestrophin R218C but were significantly altered by bestrophin W93C.
conclusionsA model of BMD was developed in the present study. Because overexpression of wt bestrophin shifted luminance response but did not alter the range of LP response amplitudes, the authors conclude that the rate-limiting step for generating LP amplitude occurs before activation of bestrophin or that bestrophin does not directly generate the LP conductance.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.