Noncell-autonomous photoreceptor degeneration in a zebrafish model of choroideremia.
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Microtubule motors transport phagosomes in the RPE, and lack of KLC1 leads to AMD-like pathogenesis.High-resolution images of retinal structure in patients with choroideremia.Loss-of-function mutations in Rab escort protein 1 (REP-1) affect intracellular transport in fibroblasts and monocytes of choroideremia patientsQuantification of retinal pigment epithelial phenotypic variation using laser scanning cytometry.Silencing of the CHM gene alters phagocytic and secretory pathways in the retinal pigment epitheliumAnalysis of the retina in the zebrafish model.Genetics of photoreceptor degeneration and regeneration in zebrafish.Retinal pigment epithelium defects accelerate photoreceptor degeneration in cell type-specific knockout mouse models of choroideremiaGene therapy for choroideremia using an adeno-associated viral (AAV) vector.Toward a better understanding of human eye disease insights from the zebrafish, Danio rerio.Proof of concept for AAV2/5-mediated gene therapy in iPSC-derived retinal pigment epithelium of a choroideremia patient.Molecular analysis of the choroideremia gene related clinical findings in two families with choroideremia.The visual system of zebrafish and its use to model human ocular diseases.Pathogenic mechanisms and the prospect of gene therapy for choroideremia.Transition zones between healthy and diseased retina in choroideremia (CHM) and Stargardt disease (STGD) as compared to retinitis pigmentosa (RP)Zebrafish: a model system for the study of eye genetics.Choroideremia: analysis of the retina from a female symptomatic carrier.Age-related cone abnormalities in zebrafish with genetic lesions in sonic hedgehog.Choroideremia: new findings from ocular pathology and review of recent literature.Arl13b Interacts With Vangl2 to Regulate Cilia and Photoreceptor Outer Segment Length in Zebrafish.Diagnosis for choroideremia in a large Chinese pedigree by next‑generation sequencing (NGS) and non‑invasive prenatal testing (NIPT).REP1 inhibits FOXO3-mediated apoptosis to promote cancer cell survivalOncogenic role of rab escort protein 1 through EGFR and STAT3 pathwayInvestigating the genetics of visual processing, function and behaviour in zebrafish.The genetics of ocular disorders: insights from the zebrafish.Choroideremia: a review of general findings and pathogenesis.Structure, regulation and cellular functions of Rab geranylgeranyl transferase and its cellular partner Rab Escort Protein.REP1 Modulates Autophagy and Macropinocytosis to Enhance Cancer Cell Survival.GUCA1A mutation causes maculopathy in a five-generation family with a wide spectrum of severity.Early stages of retinal development depend on Sec13 function.Photoreceptor-induced RPE phagolysosomal maturation defects in Stargardt-like Maculopathy (STGD3).The effect of PTC124 on choroideremia fibroblasts and iPSC-derived RPE raises considerations for therapy.
P2860
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P2860
Noncell-autonomous photoreceptor degeneration in a zebrafish model of choroideremia.
description
2007 nî lūn-bûn
@nan
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
2007年论文
@zh
2007年论文
@zh-cn
name
Noncell-autonomous photoreceptor degeneration in a zebrafish model of choroideremia.
@ast
Noncell-autonomous photoreceptor degeneration in a zebrafish model of choroideremia.
@en
type
label
Noncell-autonomous photoreceptor degeneration in a zebrafish model of choroideremia.
@ast
Noncell-autonomous photoreceptor degeneration in a zebrafish model of choroideremia.
@en
prefLabel
Noncell-autonomous photoreceptor degeneration in a zebrafish model of choroideremia.
@ast
Noncell-autonomous photoreceptor degeneration in a zebrafish model of choroideremia.
@en
P2093
P2860
P356
P1476
Noncell-autonomous photoreceptor degeneration in a zebrafish model of choroideremia.
@en
P2093
Brian D Perkins
Bryan L Krock
Joseph Bilotta
P2860
P304
P356
10.1073/PNAS.0605818104
P407
P577
2007-03-05T00:00:00Z