Associated Papers
Modifiers and their impact on inherited retinal diseases: a review.
Ford LM et al.
Ophthalmic Genetics, 2025
Retinitis Pigmentosa: Novel Therapeutic Targets and Drug Development.
Wu KY et al.
Pharmaceutics, 2023
Rhodopsin-associated retinal dystrophy: Disease mechanisms and therapeutic strategies.
Zhen F et al.
Frontiers in Neuroscience, 2023
Molecular basis for variations in the sensitivity of pathogenic rhodopsin variants to 9-cis-retinal.
Roushar FJ et al.
The Journal of Biological Chemistry, 2022
Deficiency in Retinal TGFβ Signaling Aggravates Neurodegeneration by Modulating Pro-Apoptotic and MAP Kinase Pathways.
Bielmeier CB et al.
International Journal of Molecular Sciences, 2022
Transcriptional Profiling Identifies Upregulation of Neuroprotective Pathways in Retinitis Pigmentosa.
Bielmeier CB et al.
International Journal of Molecular Sciences, 2021
Characterizing variants of unknown significance in rhodopsin: A functional genomics approach.
Wan A et al.
Human Mutation, 2019
Coupling of Human Rhodopsin to a Yeast Signaling Pathway Enables Characterization of Mutations Associated with Retinal Disease.
Scott BM et al.
Genetics, 2019
Sector retinitis pigmentosa: Report of ten cases and a review of the literature.
Coussa RG et al.
Molecular Vision, 2019
Phenotypic characterization of P23H and S334ter rhodopsin transgenic rat models of inherited retinal degeneration.
LaVail MM et al.
Experimental Eye Research, 2018
The molecular and cellular basis of rhodopsin retinitis pigmentosa reveals potential strategies for therapy.
Athanasiou D et al.
Progress in Retinal and Eye Research, 2018
Effects of Pathogenic Variations in the Human Rhodopsin Gene (hRHO) on the Predicted Accessibility for a Lead Candidate Ribozyme.
Froebel BR et al.
Investigative Ophthalmology & Visual Science, 2017
Ablation of Chop Transiently Enhances Photoreceptor Survival but Does Not Prevent Retinal Degeneration in Transgenic Mice Expressing Human P23H Rhodopsin.
Chiang WC et al.
Advances in Experimental Medicine and Biology, 2016
Genetic modifiers and oligogenic inheritance.
Kousi M et al.
Cold Spring Harbor Perspectives in Medicine, 2015
Inherent instability of the retinitis pigmentosa P23H mutant opsin.
Chen Y et al.
The Journal of Biological Chemistry, 2014
Assessing the correlation between mutant rhodopsin stability and the severity of retinitis pigmentosa.
McKeone R et al.
Molecular Vision, 2014
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 pigmentosa.
Adekeye A et al.
Plos One, 2014
Endoplasmic reticulum stress in vertebrate mutant rhodopsin models of retinal degeneration.
Kroeger H et al.
Advances in Experimental Medicine and Biology, 2014
Retinitis pigmentosa mutants provide insight into the role of the N-terminal cap in rhodopsin folding, structure, and function.
Opefi CA et al.
The Journal of Biological Chemistry, 2013
Dysmorphic photoreceptors in a P23H mutant rhodopsin model of retinitis pigmentosa are metabolically active and capable of regenerating to reverse retinal degeneration.
Lee DC et al.
The Journal of Neuroscience : the Official Journal of the Society for Neuroscience, 2012
Rhodopsin gene expression determines rod outer segment size and rod cell resistance to a dominant-negative neurodegeneration mutant.
Price BA et al.
Plos One, 2012
Mislocalization and degradation of human P23H-rhodopsin-GFP in a knockin mouse model of retinitis pigmentosa.
Price BA et al.
Investigative Ophthalmology & Visual Science, 2011
ER stress in retinal degeneration: a target for rational therapy?
Griciuc A et al.
Trends in Molecular Medicine, 2011
Retinal degeneration modulates intracellular localization of CDC42 in photoreceptors.
Heynen SR et al.
Molecular Vision, 2011
Inactivation of VCP/ter94 suppresses retinal pathology caused by misfolded rhodopsin in Drosophila.
Griciuc A et al.
Plos Genetics, 2010
High levels of retinal docosahexaenoic acid do not protect photoreceptor degeneration in VPP transgenic mice.
Li F et al.
Molecular Vision, 2010
Enhanced retinal insulin receptor-activated neuroprotective survival signal in mice lacking the protein-tyrosine phosphatase-1B gene.
Rajala RV et al.
The Journal of Biological Chemistry, 2010
In vitro analysis of ribozyme-mediated knockdown of an ADRP associated rhodopsin mutation.
Chakraborty D et al.
Advances in Experimental Medicine and Biology, 2008
A novel form of transducin-dependent retinal degeneration: accelerated retinal degeneration in the absence of rod transducin.
Brill E et al.
Investigative Ophthalmology & Visual Science, 2007
Inactivating mutations of G protein-coupled receptors and diseases: structure-function insights and therapeutic implications.
Tao YX
Pharmacology & Therapeutics, 2006
Light and inherited retinal degeneration.
Paskowitz DM et al.
The British Journal of Ophthalmology, 2006
Rpe65 as a modifier gene for inherited retinal degeneration.
Samardzija M et al.
The European Journal of Neuroscience, 2006
Novel rhodopsin mutations and genotype-phenotype correlation in patients with autosomal dominant retinitis pigmentosa.
Schuster A et al.
The British Journal of Ophthalmology, 2005
A complete screen for mutations of the rhodopsin gene in a panel of Chinese patients with autosomal dominant retinitis pigmentosa.
Zhang XL et al.
Chinese Medical Sciences Journal = Chung-kuo I Hsueh K'o Hsueh Tsa Chih, 2005
The nature of dominant mutations of rhodopsin and implications for gene therapy.
Wilson JH et al.
Molecular Neurobiology, 2003
Retinal remodeling triggered by photoreceptor degenerations.
Jones BW et al.
The Journal of Comparative Neurology, 2003
Mouse models of human retinal disease caused by expression of mutant rhodopsin. A valuable tool for the assessment of novel gene therapies.
Nour M et al.
Advances in Experimental Medicine and Biology, 2003
Deficiency of rds/peripherin causes photoreceptor death in mouse models of digenic and dominant retinitis pigmentosa.
Kedzierski W et al.
Proceedings of the National Academy of Sciences of the United States of America, 2001
Pathways to photoreceptor cell death in inherited retinal degenerations.
Pierce EA
Bioessays : News and Reviews in Molecular, Cellular and Developmental Biology, 2001
Mutant rhodopsin transgene expression on a null background.
Frederick JM et al.
Investigative Ophthalmology & Visual Science, 2001
Rhodopsin trafficking and its role in retinal dystrophies.
Sung CH et al.
International Review of Cytology, 2000
[Genotype-phenotype correlation in Chinese patients with retinitis pigmentosa due to rhodopsin mutation].
Zhang Q et al.
Yan Ke Xue Bao = Eye Science, 1999
Opsin localization and rhodopsin photochemistry in a transgenic mouse model of retinitis pigmentosa.
Wu TH et al.
Neuroscience, 1998
Polygenic disease and retinitis pigmentosa: albinism exacerbates photoreceptor degeneration induced by the expression of a mutant opsin in transgenic mice.
Naash MI et al.
The Journal of Neuroscience : the Official Journal of the Society for Neuroscience, 1996
Light-induced acceleration of photoreceptor degeneration in transgenic mice expressing mutant rhodopsin.
Naash ML et al.
Investigative Ophthalmology & Visual Science, 1996
Functional abnormalities in transgenic mice expressing a mutant rhodopsin gene.
Goto Y et al.
Investigative Ophthalmology & Visual Science, 1995
Simulation of human autosomal dominant retinitis pigmentosa in transgenic mice expressing a mutated murine opsin gene.
Naash MI et al.
Proceedings of the National Academy of Sciences of the United States of America, 1993
Expression of a mutant opsin gene increases the susceptibility of the retina to light damage.
Wang M et al.
Visual Neuroscience, 0