Document Detail


Rod-derived cone viability factor for treating blinding diseases: from clinic to redox signaling.
MedLine Citation:
PMID:  20375363     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Thierry Léveillard; José-Alain Sahel
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Science translational medicine     Volume:  2     ISSN:  1946-6242     ISO Abbreviation:  Sci Transl Med     Publication Date:  2010 Apr 
Date Detail:
Created Date:  2010-04-08     Completed Date:  2010-09-20     Revised Date:  2010-10-04    
Medline Journal Info:
Nlm Unique ID:  101505086     Medline TA:  Sci Transl Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  26ps16     Citation Subset:  IM    
Affiliation:
Department of Genetics, Institut de la Vision, INSERM, UPMC University of Paris 06, UMR-S 968, CNRS 7210, Paris F-75012, France. thierry.leveillard@inserm.fr
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MeSH Terms
Descriptor/Qualifier:
Animals
Blindness / therapy*
Cell Survival
Humans
Mice
Oxidation-Reduction
Oxidative Stress
Retinal Cone Photoreceptor Cells / metabolism*
Retinal Degeneration / therapy
Retinal Diseases / therapy
Retinal Rod Photoreceptor Cells / metabolism*
Signal Transduction*
Thioredoxins / genetics,  metabolism*
Translational Research*
Chemical
Reg. No./Substance:
52500-60-4/Thioredoxins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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