Identification and characterization of rod-derived cone viability factor

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作者
Thierry Léveillard
Saddek Mohand-Saïd
Olivier Lorentz
David Hicks
Anne-Claire Fintz
Emmanuelle Clérin
Manuel Simonutti
Valérie Forster
Nükhet Cavusoglu
Frédéric Chalmel
Pascal Dollé
Olivier Poch
George Lambrou
José-Alain Sahel
机构
[1] Laboratoire de Physiopathologie Cellulaire et Moléculaire et de la Rétine,
[2] Inserm U592,undefined
[3] Université Pierre et Marie Curie,undefined
[4] Hôpital St-Antoine,undefined
[5] 184 rue du Faubourg St-Antoine,undefined
[6] Institut de Génétique et de Biologie Moléculaire et Cellulaire,undefined
[7] 1 rue Laurent Fries,undefined
[8] Novartis Pharma AG Ophthalmology Research WKL-127.1.04,undefined
[9] Institute of Ophthalmology,undefined
[10] University College of London,undefined
来源
Nature Genetics | 2004年 / 36卷
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摘要
Retinitis pigmentosa is an untreatable, inherited retinal disease that leads to blindness. The disease initiates with the loss of night vision due to rod photoreceptor degeneration, followed by irreversible, progressive loss of cone photoreceptor1,2,3. Cone loss is responsible for the main visual handicap, as cones are essential for day and high-acuity vision4. Their loss is indirect, as most genes associated with retinitis pigmentosa are not expressed by these cells. We previously showed that factors secreted from rods are essential for cone viability5,6,7,8. Here we identified one such trophic factor by expression cloning and named it rod-derived cone viability factor (RdCVF). RdCVF is a truncated thioredoxin-like protein specifically expressed by photoreceptors. The identification of this protein offers new treatment possibilities for retinitis pigmentosa.
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页码:755 / 759
页数:4
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