Modulating expression of peripherin/rds in transgenic mice: critical levels and the effect of overexpression.
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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 PhotoreceptorsA partial structural and functional rescue of a retinitis pigmentosa model with compacted DNA nanoparticlesDifferential requirements for retinal degeneration slow intermolecular disulfide-linked oligomerization in rods versus conesInsights 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-RDSExpression of wild-type Rp1 protein in Rp1 knock-in mice rescues the retinal degeneration phenotypeGene therapy for PRPH2-associated ocular disease: challenges and prospectsOverexpression of retinal degeneration slow (RDS) protein adversely affects rods in the rd7 model of enhanced S-cone syndromeGlycosylation 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 photoreceptorsEffect of Rds abundance on cone outer segment morphogenesis, photoreceptor gene expression, and outer limiting membrane integrityTransgenic 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 pigmentosaLight/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 miceBiochemical 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
P2860
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P2860
Modulating expression of peripherin/rds in transgenic mice: critical levels and the effect of overexpression.
description
2004 nî lūn-bûn
@nan
2004 թուականի Օգոստոսին հրատարակուած գիտական յօդուած
@hyw
2004 թվականի օգոստոսին հրատարակված գիտական հոդված
@hy
2004年の論文
@ja
2004年論文
@yue
2004年論文
@zh-hant
2004年論文
@zh-hk
2004年論文
@zh-mo
2004年論文
@zh-tw
2004年论文
@wuu
name
Modulating expression of perip ...... the effect of overexpression.
@ast
Modulating expression of perip ...... the effect of overexpression.
@en
Modulating expression of perip ...... the effect of overexpression.
@nl
type
label
Modulating expression of perip ...... the effect of overexpression.
@ast
Modulating expression of perip ...... the effect of overexpression.
@en
Modulating expression of perip ...... the effect of overexpression.
@nl
prefLabel
Modulating expression of perip ...... the effect of overexpression.
@ast
Modulating expression of perip ...... the effect of overexpression.
@en
Modulating expression of perip ...... the effect of overexpression.
@nl
P2093
P2860
P356
P1476
Modulating expression of perip ...... the effect of overexpression.
@en
P2093
Heidi Stricker
Muna I Naash
Steven J Fliesler
Xi-Qin Ding
P2860
P304
P356
10.1167/IOVS.04-0065
P407
P577
2004-08-01T00:00:00Z