Loss of Daylight Vision in Retinal Degeneration: Are Oxidative Stress and Metabolic Dysregulation to Blame?

被引:138
|
作者
Punzo, Claudio [3 ,4 ]
Xiong, Wenjun [1 ,2 ]
Cepko, Constance L. [1 ,2 ]
机构
[1] Harvard Univ, Sch Med, Howard Hughes Med Inst, Dept Genet, Boston, MA 02115 USA
[2] Harvard Univ, Sch Med, Howard Hughes Med Inst, Dept Ophthalmol, Boston, MA 02115 USA
[3] Univ Massachusetts, Sch Med, Dept Ophthalmol, Worcester, MA 01606 USA
[4] Univ Massachusetts, Sch Med, Gene Therapy Ctr, Worcester, MA 01606 USA
关键词
CONE CELL-DEATH; NEUROPROTECTIVE SURVIVAL SIGNAL; RESTORES VISUAL RESPONSES; PHOTORECEPTOR CELLS; GENE-THERAPY; RETINITIS-PIGMENTOSA; MICE LACKING; PHOSPHOINOSITIDE; 3-KINASE; GLUTATHIONE-PEROXIDASE; INCREASED EXPRESSION;
D O I
10.1074/jbc.R111.304428
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Retinitis pigmentosa is characterized by loss of night vision, followed by complete blindness. Over 40 genetic loci for retinitis pigmentosa have been identified in humans, primarily affecting photoreceptor structure and function. The availability of excellent animal models allows for a mechanistic characterization of the disease. Metabolic dysregulation and oxidative stress have been found to correlate with the loss of vision, particularly in cones, the type of photoreceptors that mediate daylight and color vision. The evidence that these problems actually cause loss of vision and potential therapeutic approaches targeting them are discussed.
引用
收藏
页码:1642 / 1648
页数:7
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