Early-onset, slow progression of cone photoreceptor dysfunction and degeneration in CNG channel subunit CNGB3 deficiency.
about
AAV-mediated gene therapy in mouse models of recessive retinal degenerationGene Augmentation Therapy Restores Retinal Function and Visual Behavior in a Sheep Model of CNGA3 AchromatopsiaCNTF and retinaCNGB3-achromatopsia clinical trial with CNTF: diminished rod pathway responses with no evidence of improvement in cone functionInhibition of thyroid hormone receptor locally in the retina is a therapeutic strategy for retinal degeneration.Long-term and age-dependent restoration of visual function in a mouse model of CNGB3-associated achromatopsia following gene therapyCNGA3 deficiency affects cone synaptic terminal structure and function and leads to secondary rod dysfunction and degeneration.cGMP/Protein Kinase G Signaling Suppresses Inositol 1,4,5-Trisphosphate Receptor Phosphorylation and Promotes Endoplasmic Reticulum Stress in Photoreceptors of Cyclic Nucleotide-gated Channel-deficient Mice.Endoplasmic reticulum stress-associated cone photoreceptor degeneration in cyclic nucleotide-gated channel deficiency.A new mouse model for stationary night blindness with mutant Slc24a1 explains the pathophysiology of the associated human disease.Intravitreal Ciliary Neurotrophic Factor Transiently Improves Cone-Mediated Function in a CNGB3-/- Mouse Model of Achromatopsia.The B3 Subunit of the Cone Cyclic Nucleotide-gated Channel Regulates the Light Responses of Cones and Contributes to the Channel Structural FlexibilityLoss of cone cyclic nucleotide-gated channel leads to alterations in light response modulating system and cellular stress response pathways: a gene expression profiling study.cGMP accumulation causes photoreceptor degeneration in CNG channel deficiency: evidence of cGMP cytotoxicity independently of enhanced CNG channel function.Cones respond to light in the absence of transducin β subunit.Rearrangement of the cone mosaic in the retina of the rat model of retinitis pigmentosaSuppressing thyroid hormone signaling preserves cone photoreceptors in mouse models of retinal degeneration.Gene replacement therapy for retinal CNG channelopathies.Gene therapy for achromatopsia.Targeting iodothyronine deiodinases locally in the retina is a therapeutic strategy for retinal degeneration.Endoplasmic reticulum (ER) Ca2+-channel activity contributes to ER stress and cone death in cyclic nucleotide-gated channel deficiency.Retinal Cyclic Nucleotide-Gated Channels: From Pathophysiology to Therapy.
P2860
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P2860
Early-onset, slow progression of cone photoreceptor dysfunction and degeneration in CNG channel subunit CNGB3 deficiency.
description
2011 nî lūn-bûn
@nan
2011 թուականի Մայիսին հրատարակուած գիտական յօդուած
@hyw
2011 թվականի մայիսին հրատարակված գիտական հոդված
@hy
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
name
Early-onset, slow progression ...... nnel subunit CNGB3 deficiency.
@ast
Early-onset, slow progression ...... nnel subunit CNGB3 deficiency.
@en
type
label
Early-onset, slow progression ...... nnel subunit CNGB3 deficiency.
@ast
Early-onset, slow progression ...... nnel subunit CNGB3 deficiency.
@en
prefLabel
Early-onset, slow progression ...... nnel subunit CNGB3 deficiency.
@ast
Early-onset, slow progression ...... nnel subunit CNGB3 deficiency.
@en
P2093
P2860
P356
P1476
Early-onset, slow progression ...... nnel subunit CNGB3 deficiency.
@en
P2093
David M Sherry
Jianhua Xu
Lynsie Morris
Steven J Fliesler
Xi-Qin Ding
P2860
P304
P356
10.1167/IOVS.10-6358
P407
P577
2011-05-01T00:00:00Z