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Secretory defect and cytotoxicity: the potential disease mechanisms for the retinitis pigmentosa (RP)-associated interphotoreceptor retinoid-binding protein (IRBP)Functional characterization of an AQP0 missense mutation, R33C, that causes dominant congenital lens cataract, reveals impaired cell-to-cell adhesionTranslational control of gene expression in the gonadotropeA phenotypic high throughput screening assay for the identification of pharmacoperones for the gonadotropin releasing hormone receptorSmoke exposure causes endoplasmic reticulum stress and lipid accumulation in retinal pigment epithelium through oxidative stress and complement activation.Formation of lipofuscin-like material in the RPE Cell by different components of rod outer segments.Chaperoning G protein-coupled receptors: from cell biology to therapeuticsEndoplasmic reticulum stress and the unfolded protein responses in retinal degeneration.Endoplasmic reticulum stress is chronically activated in chronic pancreatitisRetinal degeneration in two lines of transgenic S334ter ratsGenetic ablation of N-linked glycosylation reveals two key folding pathways for R345W fibulin-3, a secreted protein associated with retinal degeneration.Autophagy supports survival and phototransduction protein levels in rod photoreceptors.Novel VCP modulators mitigate major pathologies of rd10, a mouse model of retinitis pigmentosa.Assay strategies for identification of therapeutic leads that target protein traffickingATF6 Is Mutated in Early Onset Photoreceptor Degeneration With Macular Involvement.Impact of Subunit Composition on the Uptake of α-Crystallin by Lens and RetinaThe unfolded protein response is required for dendrite morphogenesis.Computational molecular phenotyping of retinal sheet transplants to rats with retinal degeneration.Propofol Decreases Endoplasmic Reticulum Stress-Mediated Apoptosis in Retinal Pigment Epithelial Cells.Light Induces Ultrastructural Changes in Rod Outer and Inner Segments, Including Autophagy, in a Transgenic Xenopus laevis P23H Rhodopsin Model of Retinitis Pigmentosa.Induction of endoplasmic reticulum stress genes, BiP and chop, in genetic and environmental models of retinal degenerationP23H opsin knock-in mice reveal a novel step in retinal rod disc morphogenesis.Inherent instability of the retinitis pigmentosa P23H mutant opsin.Gene-based therapies for dominantly inherited retinopathies.An overview of transcriptional regulation in response to toxicological insult.Gender specific issues in hereditary ocular disorders.Small molecule proteostasis regulators that reprogram the ER to reduce extracellular protein aggregation.Mouse Models of Stargardt 3 Dominant Macular DegenerationAAV Delivery of GRP78/BiP Promotes Adaptation of Human RPE Cell to ER Stress.Protective effect of clusterin on rod photoreceptor in rat model of retinitis pigmentosaNeuoroprotective efficacies by KUS121, a VCP modulator, on animal models of retinal degeneration.Activation of TGF-β signaling induces cell death via the unfolded protein response in Fuchs endothelial corneal dystrophy.Phenotypic characterization of P23H and S334ter rhodopsin transgenic rat models of inherited retinal degeneration.Dark Rearing Does Not Prevent Rod Oxidative Stress In Vivo in Pde6brd10 Mice.Perspective of Future Potent Therapies for Fuchs Endothelial Corneal Dystrophy
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P2860
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on January 2010
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
Misfolded proteins and retinal dystrophies.
@en
Misfolded proteins and retinal dystrophies.
@nl
type
label
Misfolded proteins and retinal dystrophies.
@en
Misfolded proteins and retinal dystrophies.
@nl
prefLabel
Misfolded proteins and retinal dystrophies.
@en
Misfolded proteins and retinal dystrophies.
@nl
P2860
P1476
Misfolded proteins and retinal dystrophies.
@en
P2093
Jonathan H Lin
Matthew M Lavail
P2860
P304
P356
10.1007/978-1-4419-1399-9_14
P407
P577
2010-01-01T00:00:00Z