Rod-Derived Cone Viability Factor for Treating Blinding Diseases: From Clinic to Redox Signaling

被引：99

作者：

Leveillard, Thierry ^{[1
]}

Sahel, Jose-Alain ^{[1
,2
,3
,4
,5
,6
]}

机构：

[1] Univ Paris 06, UPMC, CNRS 7210, INSERM,Dept Genet,Inst Vis,UMR S 968, F-75012 Paris, France

[2] Univ Paris 06, Dept Therapeut, CNRS 7210, INSERM,UPMC,Inst Vis,UMR S 968, F-75012 Paris, France

[3] Serv Pr Sahel, Ctr Hosp Natl Ophtalmol Quinze Vingts, Paris, France

[4] INSERM DHOS CIC 503, Paris, France

[5] UCL, Inst Ophthalmol, London WC1E 6BT, England

[6] Fdn Ophtalmol Adolphe Rothschild, Paris, France

来源：

SCIENCE TRANSLATIONAL MEDICINE | 2010年 / 2卷 / 26期

关键词：

LEBERS CONGENITAL AMAUROSIS; THIOREDOXIN-LIKE PROTEIN; RETINITIS-PIGMENTOSA; GENE-THERAPY; RETINAL DEGENERATION; MODEL; RPE65; MOUSE; DEATH; TRANSPLANTATION;

D O I：

10.1126/scitranslmed.3000866

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

The identification of one mechanism that causes vision loss in inherited degenerative retinal disorders revealed a new signaling molecule that represents a potential therapy for these currently untreatable diseases. This protein, called rod-derived cone viability factor (RdCVF), maintains the function and consequently the viability of cone photoreceptor cells in the retina; mice that lack this factor exhibit a progressive loss of photoreceptor cells. The gene encoding RdCVF also encodes, by differential splicing, a second product that has characteristics of a thioredoxin-like enzyme and protects both photoreceptor cells and, more specifically, its interacting protein partner, the tau protein, against oxidative damage. This signaling pathway potentially links environmental insults to an endogenous neuroprotective response.

引用

页数：5

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