Restoration of visual function in P23H rhodopsin transgenic rats by gene delivery of BiP/Grp78
about
Secretory defect and cytotoxicity: the potential disease mechanisms for the retinitis pigmentosa (RP)-associated interphotoreceptor retinoid-binding protein (IRBP)Pharmacoperones: a new therapeutic approach for diseases caused by misfolded G protein-coupled receptorsA comprehensive review of retinal gene therapyDefining the catalytic activity of nanoceria in the P23H-1 rat, a photoreceptor degeneration modelAblation of C/EBP homologous protein does not protect T17M RHO mice from retinal degenerationInactivation of VCP/ter94 suppresses retinal pathology caused by misfolded rhodopsin in DrosophilaLimited ATF4 Expression in Degenerating Retinas with Ongoing ER Stress Promotes Photoreceptor Survival in a Mouse Model of Autosomal Dominant Retinitis PigmentosaER stress and apoptosis: a new mechanism for retinal cell death.Pharmacological modulation of the retinal unfolded protein response in Bardet-Biedl syndrome reduces apoptosis and preserves light detection abilityPotential of Small Molecule-Mediated Reprogramming of Rod Photoreceptors to Treat Retinitis PigmentosaPhenotypic assays identify azoramide as a small-molecule modulator of the unfolded protein response with antidiabetic activityHearing impairment in the P23H-1 retinal degeneration rat model.The human rhodopsin kinase promoter in an AAV5 vector confers rod- and cone-specific expression in the primate retina.The use of induced pluripotent stem cells to reveal pathogenic gene mutations and explore treatments for retinitis pigmentosa.Modulation of cellular signaling pathways in P23H rhodopsin photoreceptors.Modulation of the rate of retinal degeneration in T17M RHO mice by reprogramming the unfolded protein response.Chaperoning G protein-coupled receptors: from cell biology to therapeuticsEndoplasmic reticulum stress and the unfolded protein responses in retinal degeneration.Gene augmentation for adRP mutations in RHO.Suppression and replacement gene therapy for autosomal dominant disease in a murine model of dominant retinitis pigmentosaER stress in retinal degeneration in S334ter Rho rats.The biochemistry, ultrastructure, and subunit assembly mechanism of AMPA receptors.Induction of the unfolded protein response by constitutive G-protein signaling in rod photoreceptor cells.Rhodopsin gene expression determines rod outer segment size and rod cell resistance to a dominant-negative neurodegeneration mutantInhibition of thyroid hormone receptor locally in the retina is a therapeutic strategy for retinal degeneration.Allosteric inhibition of the IRE1α RNase preserves cell viability and function during endoplasmic reticulum stress.Inhibitory peptide of mitochondrial μ-calpain protects against photoreceptor degeneration in rhodopsin transgenic S334ter and P23H rats.Retinitis pigmentosa: genes and disease mechanisms.AAV delivery of wild-type rhodopsin preserves retinal function in a mouse model of autosomal dominant retinitis pigmentosa.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.Performance of protein disorder prediction programs on amino acid substitutions.Protein folding and quality control in the endoplasmic reticulum: Recent lessons from yeast and mammalian cell systems.Pharmacological chaperones for misfolded gonadotropin-releasing hormone receptorsIntermittent but not constant high glucose induces ER stress and inflammation in human retinal pericytesGenotypic and phenotypic characterization of P23H line 1 rat model.An activated unfolded protein response promotes retinal degeneration and triggers an inflammatory response in the mouse retina.Small-molecule proteostasis regulators for protein conformational diseases.IRE1 directs proteasomal and lysosomal degradation of misfolded rhodopsinGlucose-regulated protein 78 may play a crucial role in promoting the pulmonary microvascular remodeling in a rat model of hepatopulmonary syndrome
P2860
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P2860
Restoration of visual function in P23H rhodopsin transgenic rats by gene delivery of BiP/Grp78
description
2010 nî lūn-bûn
@nan
2010 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի մարտին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Restoration of visual function ...... by gene delivery of BiP/Grp78
@ast
Restoration of visual function ...... by gene delivery of BiP/Grp78
@en
type
label
Restoration of visual function ...... by gene delivery of BiP/Grp78
@ast
Restoration of visual function ...... by gene delivery of BiP/Grp78
@en
prefLabel
Restoration of visual function ...... by gene delivery of BiP/Grp78
@ast
Restoration of visual function ...... by gene delivery of BiP/Grp78
@en
P2093
P2860
P356
P1476
Restoration of visual function ...... by gene delivery of BiP/Grp78
@en
P2093
Jonathan H Lin
Marina S Gorbatyuk
Matthew M LaVail
Nicholas Muzyczka
Oleg S Gorbatyuk
Syed M Noorwez
Tessa Knox
P2860
P304
P356
10.1073/PNAS.0911991107
P407
P577
2010-03-15T00:00:00Z