Retinoids assist the cellular folding of the autosomal dominant retinitis pigmentosa opsin mutant P23H.
about
Therapeutic rescue of misfolded mutants: validation of primary high throughput screens for identification of pharmacoperone drugsPharmacoperone Identification for Therapeutic Rescue of Misfolded Mutant ProteinsA dual role for EDEM1 in the processing of rod opsinX-linked cone dystrophy caused by mutation of the red and green cone opsinsPharmacoperones: a new therapeutic approach for diseases caused by misfolded G protein-coupled receptorsRhodopsin mutant P23H destabilizes rod photoreceptor disk membranesFunctional role of positively selected amino acid substitutions in mammalian rhodopsin evolutionTrafficking and quality control of the gonadotropin releasing hormone receptor in health and diseaseRhodopsin: the functional significance of asn-linked glycosylation and other post-translational modificationsA phenotypic high throughput screening assay for the identification of pharmacoperones for the gonadotropin releasing hormone receptorRpe65 as a modifier gene for inherited retinal degenerationTransitioning pharmacoperones to therapeutic use: in vivo proof-of-principle and design of high throughput screens.Using pharmacological chaperones to restore proteostasisChaperoning G protein-coupled receptors: from cell biology to therapeuticsPhotoactivation-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.Probing mechanisms of photoreceptor degeneration in a new mouse model of the common form of autosomal dominant retinitis pigmentosa due to P23H opsin mutations.Exclusion of the unfolded protein response in light-induced retinal degeneration in the canine T4R RHO model of autosomal dominant retinitis pigmentosa.Molecular mechanisms of disease for mutations at Gly-90 in rhodopsinPreservation of photoreceptor morphology and function in P23H rats using an allele independent ribozyme.Pharmacological chaperones for misfolded gonadotropin-releasing hormone receptorsAssay strategies for identification of therapeutic leads that target protein traffickingEffect of rapamycin on the fate of P23H opsin associated with retinitis pigmentosa (an American Ophthalmological Society thesis).IRE1 directs proteasomal and lysosomal degradation of misfolded rhodopsinRegulation of G protein-coupled receptor export trafficking.Pharmacologic rescue of conformationally-defective proteins: implications for the treatment of human disease.Mislocalization and degradation of human P23H-rhodopsin-GFP in a knockin mouse model of retinitis pigmentosaThe melanocortin-4 receptor: physiology, pharmacology, and pathophysiology.A High-Throughput Drug Screening Strategy for Detecting Rhodopsin P23H Mutant Rescue and Degradation.M-opsin protein degradation is inhibited by MG-132 in Rpe65⁻/⁻ retinal explant cultureEndoplasmic Reticulum Stress and Unfolded Protein Response Pathways: Potential for Treating Age-related Retinal DegenerationAn S-opsin knock-in mouse (F81Y) reveals a role for the native ligand 11-cis-retinal in cone opsin biosynthesisMice with a D190N mutation in the gene encoding rhodopsin: a model for human autosomal-dominant retinitis pigmentosaLight-induced damage to the retina: role of rhodopsin chromophore revisited.Selective activation of ATF6 and PERK endoplasmic reticulum stress signaling pathways prevent mutant rhodopsin accumulationInduction of endoplasmic reticulum stress genes, BiP and chop, in genetic and environmental models of retinal degenerationClinical light exposure, photoreceptor degeneration, and AP-1 activation: a cell death or cell survival signal in the rhodopsin mutant retina?Unfolding the Therapeutic Potential of Chemical Chaperones for Age-related Macular DegenerationExplant cultures of Rpe65-/- mouse retina: a model to investigate cone opsin trafficking.Dysmorphic photoreceptors in a P23H mutant rhodopsin model of retinitis pigmentosa are metabolically active and capable of regenerating to reverse retinal degeneration.
P2860
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P2860
Retinoids assist the cellular folding of the autosomal dominant retinitis pigmentosa opsin mutant P23H.
description
2004 nî lūn-bûn
@nan
2004年の論文
@ja
2004年学术文章
@wuu
2004年学术文章
@zh
2004年学术文章
@zh-cn
2004年学术文章
@zh-hans
2004年学术文章
@zh-my
2004年学术文章
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2004年學術文章
@yue
2004年學術文章
@zh-hant
name
Retinoids assist the cellular ...... pigmentosa opsin mutant P23H.
@en
Retinoids assist the cellular ...... pigmentosa opsin mutant P23H.
@nl
type
label
Retinoids assist the cellular ...... pigmentosa opsin mutant P23H.
@en
Retinoids assist the cellular ...... pigmentosa opsin mutant P23H.
@nl
prefLabel
Retinoids assist the cellular ...... pigmentosa opsin mutant P23H.
@en
Retinoids assist the cellular ...... pigmentosa opsin mutant P23H.
@nl
P2093
P2860
P356
P1476
Retinoids assist the cellular ...... pigmentosa opsin mutant P23H.
@en
P2093
J Hugh McDowell
Karen A Smith
Ritu Malhotra
Shalesh Kaushal
Syed M Noorwez
P2860
P304
16278-16284
P356
10.1074/JBC.M312101200
P407
P577
2004-02-09T00:00:00Z