Effect of vitamin A supplementation on rhodopsin mutants threonine-17 --> methionine and proline-347 --> serine in transgenic mice and in cell cultures
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AIPL1, the protein that is defective in Leber congenital amaurosis, is essential for the biosynthesis of retinal rod cGMP phosphodiesteraseUsherin is required for maintenance of retinal photoreceptors and normal development of cochlear hair cellsA retinitis pigmentosa GTPase regulator (RPGR)-deficient mouse model for X-linked retinitis pigmentosa (RP3)Endoplasmic reticulum degradation impedes olfactory G-protein coupled receptor functional expressionPharmacological and rAAV gene therapy rescue of visual functions in a blind mouse model of Leber congenital amaurosis.Pharmacoperones: a new therapeutic approach for diseases caused by misfolded G protein-coupled receptorsLimited ATF4 Expression in Degenerating Retinas with Ongoing ER Stress Promotes Photoreceptor Survival in a Mouse Model of Autosomal Dominant Retinitis PigmentosaThe retinitis pigmentosa GTPase regulator (RPGR)- interacting protein: subserving RPGR function and participating in disk morphogenesisThe ciliary rootlet maintains long-term stability of sensory ciliaRhodopsin: the functional significance of asn-linked glycosylation and other post-translational modificationsThe methionine-aromatic motif plays a unique role in stabilizing protein structure.Increased light exposure alleviates one form of photoreceptor degeneration marked by elevated calcium in the darkNon-invasive stem cell therapy in a rat model for retinal degeneration and vascular pathology.Mutations of the opsin gene (Y102H and I307N) lead to light-induced degeneration of photoreceptors and constitutive activation of phototransduction in mice.Retinoids for treatment of retinal diseasesRetinitis pigmentosa and allied conditions today: a paradigm of translational researchChaperoning G protein-coupled receptors: from cell biology to therapeuticsRecent advances in the molecular basis of inherited photoreceptor degeneration.Naturally occurring rhodopsin mutation in the dog causes retinal dysfunction and degeneration mimicking human dominant retinitis pigmentosaP2Y(2) receptor agonist INS37217 enhances functional recovery after detachment caused by subretinal injection in normal and rds mice.Photoactivation-induced instability of rhodopsin mutants T4K and T17M in rod outer segments underlies retinal degeneration in X. laevis transgenic models of retinitis pigmentosaA naturally occurring mutation of the opsin gene (T4R) in dogs affects glycosylation and stability of the G protein-coupled receptor.Pharmacological chaperone-mediated in vivo folding and stabilization of the P23H-opsin mutant associated with autosomal dominant retinitis pigmentosaThe biochemistry, ultrastructure, and subunit assembly mechanism of AMPA receptors.Biosynthesis, characterization, and efficacy in retinal degenerative diseases of lens epithelium-derived growth factor fragment (LEDGF1-326), a novel therapeutic protein.A novel form of transducin-dependent retinal degeneration: accelerated retinal degeneration in the absence of rod transducinChemical chaperone 4-phenylbutyrate prevents endoplasmic reticulum stress induced by T17M rhodopsin.G protein-coupled receptor rhodopsin.Ablation of the proapoptotic genes CHOP or Ask1 does not prevent or delay loss of visual function in a P23H transgenic mouse model of retinitis pigmentosaExclusion of the unfolded protein response in light-induced retinal degeneration in the canine T4R RHO model of autosomal dominant retinitis pigmentosa.Drug-inducible synergistic gene silencing with multiple small hairpin RNA molecules for gene function study in animal model.Misexpression of the constitutive Rpgr(ex1-19) variant leads to severe photoreceptor degeneration.Quality of life in patients with retinitis pigmentosa submitted to intravitreal use of bone marrow-derived stem cells (Reticell -clinical trial)Glycosylation of rhodopsin is necessary for its stability and incorporation into photoreceptor outer segment discs.Overview. Predictive DNA testing in ophthalmology.IRE1 directs proteasomal and lysosomal degradation of misfolded rhodopsinTargeting Caspase-12 to Preserve Vision in Mice With Inherited Retinal Degeneration.Development of lead hammerhead ribozyme candidates against human rod opsin mRNA for retinal degeneration therapy.ER stress is involved in T17M rhodopsin-induced retinal degeneration.Diseases caused by defects in the visual cycle: retinoids as potential therapeutic agents
P2860
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P2860
Effect of vitamin A supplementation on rhodopsin mutants threonine-17 --> methionine and proline-347 --> serine in transgenic mice and in cell cultures
description
1998 nî lūn-bûn
@nan
1998年の論文
@ja
1998年学术文章
@wuu
1998年学术文章
@zh-cn
1998年学术文章
@zh-hans
1998年学术文章
@zh-my
1998年学术文章
@zh-sg
1998年學術文章
@yue
1998年學術文章
@zh
1998年學術文章
@zh-hant
name
Effect of vitamin A supplement ...... enic mice and in cell cultures
@ast
Effect of vitamin A supplement ...... enic mice and in cell cultures
@en
type
label
Effect of vitamin A supplement ...... enic mice and in cell cultures
@ast
Effect of vitamin A supplement ...... enic mice and in cell cultures
@en
prefLabel
Effect of vitamin A supplement ...... enic mice and in cell cultures
@ast
Effect of vitamin A supplement ...... enic mice and in cell cultures
@en
P2093
P2860
P356
P1476
Effect of vitamin A supplement ...... enic mice and in cell cultures
@en
P2093
B S Pawlyk
E L Berson
M A Sandberg
P2860
P304
11933-11938
P356
10.1073/PNAS.95.20.11933
P407
P577
1998-09-01T00:00:00Z