New views on RPE65 deficiency: the rod system is the source of vision in a mouse model of Leber congenital amaurosis.
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Lentiviral gene transfer of RPE65 rescues survival and function of cones in a mouse model of Leber congenital amaurosisInactivation of the murine X-linked juvenile retinoschisis gene, Rs1h, suggests a role of retinoschisin in retinal cell layer organization and synaptic structureOpsin activation of transduction in the rods of dark-reared Rpe65 knockout miceRapid degradation of dominant-negative Rab27 proteins in vivo precludes their use in transgenic mouse models.Recombinant adeno-associated virus type 2-mediated gene delivery into the Rpe65-/- knockout mouse eye results in limited rescueSynaptogenesis and outer segment formation are perturbed in the neural retina of Crx mutant miceAAV-mediated gene therapy in mouse models of recessive retinal degenerationPARP1 gene knock-out increases resistance to retinal degeneration without affecting retinal function.Dissecting a role for melanopsin in behavioural light aversion reveals a response independent of conventional photoreceptionNonvisual light responses in the Rpe65 knockout mouse: rod loss restores sensitivity to the melanopsin system.Recovery of visual functions in a mouse model of Leber congenital amaurosisImpaired channel targeting and retinal degeneration in mice lacking the cyclic nucleotide-gated channel subunit CNGB1A critical role of CaBP4 in the cone synapse.Rod-driven OFF pathway responses in the distal retina: dark-adapted flicker electroretinogram in mouseAAV-mediated cone rescue in a naturally occurring mouse model of CNGA3-achromatopsiaRetinal pathways influence temporal niche.Circadian photoreception in vertebrates.Flow of energy in the outer retina in darkness and in light.Restoration of cone vision in the CNGA3-/- mouse model of congenital complete lack of cone photoreceptor functionRetinal Thickness Normative Data in Wild-Type Mice Using Customized Miniature SD-OCT.Rb-mediated neuronal differentiation through cell-cycle-independent regulation of E2f3a.Spectral domain optical coherence tomography in mouse models of retinal degeneration.Bax-induced apoptosis in Leber's congenital amaurosis: a dual role in rod and cone degeneration.RPE65: role in the visual cycle, human retinal disease, and gene therapy.Rpe65 as a modifier gene for inherited retinal degenerationUsing the NAFX to measure the effectiveness over time of gene therapy in canine LCADeletion of GRK1 causes retina degeneration through a transducin-independent mechanism.Identifying photoreceptors in blind eyes caused by RPE65 mutations: Prerequisite for human gene therapy success.Intrinsically photosensitive retinal ganglion cells detect light with a vitamin A-based photopigment, melanopsinLight-evoked responses of the mouse retinal pigment epithelium.Leber congenital amaurosis due to RPE65 mutations and its treatment with gene therapyRetinoid processing in cone and Müller cell linesIndependent degeneration of photoreceptors and retinal pigment epithelium in conditional knockout mouse models of choroideremia.Lecithin-retinol acyltransferase is essential for accumulation of all-trans-retinyl esters in the eye and in the liver.Biochemical and physiological properties of rhodopsin regenerated with 11-cis-6-ring- and 7-ring-retinalsSelf-complementary AAV-mediated gene therapy restores cone function and prevents cone degeneration in two models of Rpe65 deficiency.Metabolic syndrome triggered by high-fructose diet favors choroidal neovascularization and impairs retinal light sensitivity in the ratEvaluation of the role of the retinal G protein-coupled receptor (RGR) in the vertebrate retina in vivo.CDC42 is required for tissue lamination and cell survival in the mouse retina.A novel role for the immunoproteasome in retinal function.
P2860
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P2860
New views on RPE65 deficiency: the rod system is the source of vision in a mouse model of Leber congenital amaurosis.
description
2001 nî lūn-bûn
@nan
2001年の論文
@ja
2001年学术文章
@wuu
2001年学术文章
@zh-cn
2001年学术文章
@zh-hans
2001年学术文章
@zh-my
2001年学术文章
@zh-sg
2001年學術文章
@yue
2001年學術文章
@zh
2001年學術文章
@zh-hant
name
New views on RPE65 deficiency: ...... of Leber congenital amaurosis.
@en
New views on RPE65 deficiency: ...... of Leber congenital amaurosis.
@nl
type
label
New views on RPE65 deficiency: ...... of Leber congenital amaurosis.
@en
New views on RPE65 deficiency: ...... of Leber congenital amaurosis.
@nl
prefLabel
New views on RPE65 deficiency: ...... of Leber congenital amaurosis.
@en
New views on RPE65 deficiency: ...... of Leber congenital amaurosis.
@nl
P2093
P50
P356
P1433
P1476
New views on RPE65 deficiency: ...... of Leber congenital amaurosis
@en
P2093
Friedburg C
Humphries P
Seeliger MW
Ståhlberg F
P2888
P356
10.1038/NG712
P407
P577
2001-09-01T00:00:00Z
P5875
P6179
1031063377