RPGR-associated retinal degeneration in human X-linked RP and a murine model.
about
Biology and therapy of inherited retinal degenerative disease: insights from mouse modelsRenal-retinal ciliopathy gene Sdccag8 regulates DNA damage response signalingSuccessful arrest of photoreceptor and vision loss expands the therapeutic window of retinal gene therapy to later stages of disease.Gene augmentation for X-linked retinitis pigmentosa caused by mutations in RPGRNatural history of cone disease in the murine model of Leber congenital amaurosis due to CEP290 mutation: determining the timing and expectation of therapy.A long-term efficacy study of gene replacement therapy for RPGR-associated retinal degenerationRPGR: Its role in photoreceptor physiology, human disease, and future therapies.Stability and Safety of an AAV Vector for Treating RPGR-ORF15 X-Linked Retinitis Pigmentosa.TULP1 mutations causing early-onset retinal degeneration: preserved but insensitive macular cones.Photoreceptor sensory cilia and ciliopathies: focus on CEP290, RPGR and their interacting proteins.AAV-mediated gene therapy in the guanylate cyclase (RetGC1/RetGC2) double knockout mouse model of Leber congenital amaurosis.Intervisit variability of visual parameters in Leber congenital amaurosis caused by RPE65 mutationsPhotoreceptor rescue by an abbreviated human RPGR gene in a murine model of X-linked retinitis pigmentosa.Ciliopathy-associated protein CEP290 modifies the severity of retinal degeneration due to loss of RPGR.RPGR-associated retinopathy: clinical features, molecular genetics, animal models and therapeutic options.Variegated yet non-random rod and cone photoreceptor disease patterns in RPGR-ORF15-associated retinal degeneration.Gene and cell-based therapies for inherited retinal disorders: An update.Gelsolin dysfunction causes photoreceptor loss in induced pluripotent cell and animal retinitis pigmentosa models.Prenylated retinal ciliopathy protein RPGR interacts with PDE6δ and regulates ciliary localization of Joubert syndrome-associated protein INPP5E.Retinitis pigmentosa caused by mutations in the ciliary MAK gene is relatively mild and is not associated with apparent extra-ocular features.Gene therapy and genome surgery in the retina.RPGR protein complex regulates proteasome activity and mediates store-operated calcium entry.Characterization of Visual Function, Interocular Variability and Progression Using Static Perimetry-Derived Metrics in RPGR-Associated Retinopathy.
P2860
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P2860
RPGR-associated retinal degeneration in human X-linked RP and a murine model.
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
2012年论文
@zh
2012年论文
@zh-cn
name
RPGR-associated retinal degeneration in human X-linked RP and a murine model.
@ast
RPGR-associated retinal degeneration in human X-linked RP and a murine model.
@en
type
label
RPGR-associated retinal degeneration in human X-linked RP and a murine model.
@ast
RPGR-associated retinal degeneration in human X-linked RP and a murine model.
@en
prefLabel
RPGR-associated retinal degeneration in human X-linked RP and a murine model.
@ast
RPGR-associated retinal degeneration in human X-linked RP and a murine model.
@en
P2093
P2860
P50
P356
P1476
RPGR-associated retinal degeneration in human X-linked RP and a murine model.
@en
P2093
Alan F Wright
Alejandro J Roman
Artur V Cideciyan
Dina Y Gewaily
Maria P Limberis
Peter Bell
Sam Sadigh
Sharon B Schwartz
Wei Chieh Huang
P2860
P304
P356
10.1167/IOVS.12-10070
P407
P577
2012-08-15T00:00:00Z