Modulating expression of peripherin/rds in transgenic mice: critical levels and the effect of overexpression. - Wikidata - awgldk - TriplyDB

Modulating expression of peripherin/rds in transgenic mice: critical levels and the effect of overexpression.

Modulating expression of peripherin/rds in transgenic mice: critical levels and the effect of overexpression.

http://www.wikidata.org/entity/Q34119855

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In Vivo Analysis of Disease-Associated Point Mutations Unveils Profound Differences in mRNA Splicing of Peripherin-2 in Rod and Cone Photoreceptors A partial structural and functional rescue of a retinitis pigmentosa model with compacted DNA nanoparticles Differential requirements for retinal degeneration slow intermolecular disulfide-linked oligomerization in rods versus cones Insights into the mechanisms of macular degeneration associated with the R172W mutation in RDS.Role of the second intradiscal loop of peripherin/rds in homo and hetero associations.Differential distribution of proteins and lipids in detergent-resistant and detergent-soluble domains in rod outer segment plasma membranes and disks.Initiation of rod outer segment disc formation requires RDS.Peripherin-2 and Rom-1 have opposing effects on rod outer segment targeting of retinitis pigmentosa-linked peripherin-2 mutants.Gene delivery to mitotic and postmitotic photoreceptors via compacted DNA nanoparticles results in improved phenotype in a mouse model of retinitis pigmentosa.Rim formation is not a prerequisite for distribution of cone photoreceptor outer segment proteins.A 350 bp region of the proximal promoter of Rds drives cell-type specific gene expression.Differences in RDS trafficking, assembly and function in cones versus rods: insights from studies of C150S-RDS Expression of wild-type Rp1 protein in Rp1 knock-in mice rescues the retinal degeneration phenotype Gene therapy for PRPH2-associated ocular disease: challenges and prospects Overexpression of retinal degeneration slow (RDS) protein adversely affects rods in the rd7 model of enhanced S-cone syndrome Glycosylation of rhodopsin is necessary for its stability and incorporation into photoreceptor outer segment discs.Direct gene transfer with compacted DNA nanoparticles in retinal pigment epithelial cells: expression, repeat delivery and lack of toxicity.Retention of function without normal disc morphogenesis occurs in cone but not rod photoreceptors Effect of Rds abundance on cone outer segment morphogenesis, photoreceptor gene expression, and outer limiting membrane integrity Transgenic animal studies of human retinal disease caused by mutations in peripherin/rds.PRPH2/RDS and ROM-1: Historical context, current views and future considerations.IMP dehydrogenase-linked retinitis pigmentosa.Outer segment oligomerization of Rds: evidence from mouse models and subcellular fractionation.Genetic supplementation of RDS alleviates a loss-of-function phenotype in C214S model of retinitis pigmentosa Light/dark translocation of alphatransducin in mouse photoreceptor cells expressing G90D mutant opsin.Gene therapy in the Retinal Degeneration Slow model of retinitis pigmentosa.Nanoparticles for retinal gene therapy.The K153Del PRPH2 mutation differentially impacts photoreceptor structure and function.Late-onset cone photoreceptor degeneration induced by R172W mutation in Rds and partial rescue by gene supplementation.The Cys214-->Ser mutation in peripherin/rds causes a loss-of-function phenotype in transgenic mice Biochemical analysis of phenotypic diversity associated with mutations in codon 244 of the retinal degeneration slow gene.Photoreceptor discs form through peripherin-dependent suppression of ciliary ectosome release.Oligomerization of Prph2 and Rom1 is essential for photoreceptor outer segment formation

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P2860

Modulating expression of peripherin/rds in transgenic mice: critical levels and the effect of overexpression.

http://www.wikidata.org/entity/Q34119855

description

2004 nî lūn-bûn

@nan

2004 թուականի Օգոստոսին հրատարակուած գիտական յօդուած

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2004 թվականի օգոստոսին հրատարակված գիտական հոդված

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2004年の論文

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2004年論文

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2004年論文

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2004年論文

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2004年論文

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2004年論文

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2004年论文

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Modulating expression of perip ...... the effect of overexpression.

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Modulating expression of perip ...... the effect of overexpression.

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Modulating expression of perip ...... the effect of overexpression.

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Modulating expression of perip ...... the effect of overexpression.

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Investigative Ophthalmology & Visual Science

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Modulating expression of perip ...... the effect of overexpression.

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P2093

Heidi Stricker

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May Nour

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Muna I Naash

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Steven J Fliesler

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Xi-Qin Ding

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P2860

Photoreceptor renewal: a role for peripherin/rds

Transgenic Bcl-2 expressed in photoreceptor cells confers both death-sparing and death-inducing effects.

Molecular characterization of the skate peripherin/rds gene: relationship to its orthologues and paralogues

The relationship between opsin overexpression and photoreceptor degeneration.

Disulfide-mediated oligomerization of Peripherin/Rds and Rom-1 in photoreceptor disk membranes. Implications for photoreceptor outer segment morphogenesis and degeneration.

Expression of cone-photoreceptor-specific antigens in a cell line derived from retinal tumors in transgenic mice

Molecular cloning, primary structure, and orientation of the vertebrate photoreceptor cell protein peripherin in the rod outer segment disk membrane.

Simulation of human autosomal dominant retinitis pigmentosa in transgenic mice expressing a mutated murine opsin gene.

Deficiency of rds/peripherin causes photoreceptor death in mouse models of digenic and dominant retinitis pigmentosa

Extensive intrafamilial and interfamilial phenotypic variation among patients with autosomal dominant retinal dystrophy and mutations in the human RDS/peripherin gene

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2514-2521

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10.1167/IOVS.04-0065

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8

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45

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8433236

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15277471

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2937570

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