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首页> 外文期刊>Journal of Cellular Physiology >Oxidative damage is a potential cause of cone cell death in retinitis pigmentosa.
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Oxidative damage is a potential cause of cone cell death in retinitis pigmentosa.

机译:视锥细胞的氧化损伤是一个潜在的原因死亡在色素性视网膜炎。

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摘要

Retinitis pigmentosa (RP) is a prevalent cause of blindness caused by a large number of different mutations in many different genes. The mutations result in rod photoreceptor cell death, but it is unknown why cones die. In this study, we tested the hypothesis that cones die from oxidative damage by performing immunohistochemical staining for biomarkers of oxidative damage in a transgenic pig model of RP. The presence of acrolein- and 4-hydroxynonenal-adducts on proteins is a specific indicator that lipid peroxidation has occurred, and there was strong immunofluorescent staining for both in cone inner segments (IS) of two 10-month-old transgenic pigs in which almost all rods had died, compared to faint staining in two 10-month-old control pig retinas. In 22- and 24-month-old transgenic pigs in which all rods and many cones had died, staining was strong in cone axons and some cell bodies as well as IS indicating progression in oxidative damage between 10 and 22 months. Biomarkers for oxidative damage to proteins and DNA also showed progressive oxidative damage to those macromolecules in cones during the course of RP. These data support the hypothesis that the death of rods results in decreased oxygen consumption and hyperoxia in the outer retina resulting in gradual cone cell death from oxidative damage. This hypothesis has important therapeutic implications and deserves rapid evaluation.
机译:视网膜色素变性(RP)是一种普遍的原因失明造成大量的不同在许多不同的基因突变。导致视杆细胞死亡,但它是未知的视锥细胞死亡的原因。锥死于氧化的假设进行免疫组织化学染色的影响氧化损伤的生物标志物转基因猪的RP模型。丙烯醛,4-hydroxynonenal-adducts脂质蛋白是一个具体的指标发生过氧化反应,强大在锥内部immunofluorescent染色段(是)的两个10个月大的转基因猪几乎所有的棒已经死亡,相比微弱的染色在两个10个月大的控制猪视网膜。所有的棒和许多锥死了,染色是在锥轴突和一些细胞身体以及显示进展氧化损伤10至22个月。生物标记蛋白质和氧化损伤DNA还显示进步的氧化损伤这些大分子在锥RP。棒的死亡导致减少氧气消费和视网膜外层氧过多导致视锥细胞逐渐死亡氧化损伤。和值得快速治疗的影响评估。

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