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首页> 外文期刊>Molecular and Cellular Biology >Retinal Degeneration and Ionizing Radiation Hypersensitivity in a Mouse Model for Cockayne Syndrome
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Retinal Degeneration and Ionizing Radiation Hypersensitivity in a Mouse Model for Cockayne Syndrome

机译：视网膜退化和电离辐射超敏反应的Cockayne综合征小鼠模型中。

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Mutations in the CSB gene cause Cockayne syndrome (CS), a DNA repair disorder characterized by UV sensitivity and severe physical and neurological impairment. CSB functions in the transcription-coupled repair subpathway of nucleotide excision repair. This function may explain the UV sensitivity but hardly clarifies the other CS symptoms. Many of these, including retinopathy, are associated with premature aging. We studied eye pathology in a mouse model for CS. Csbm/m mice were hypersensitive to UV light and developed epithelial hyperplasia and squamous cell carcinomas in the cornea, which underscores the importance of transcription-coupled repair of photolesions in the mouse. In addition, we observed a spontaneous loss of retinal photoreceptor cells with age in the Csbm/m retina, resulting in a 60% decrease in the number of rods by the age of 18 months. Importantly, when Csbm/m mice (as well as Csa?/? mice) were exposed to 10 Gy of ionizing radiation, we noticed an increase in apoptotic photoreceptor cells, which was not observed in wild-type animals. This finding, together with our observation that the expression of established oxidative stress marker genes is upregulated in the Csbm/m retina, suggests that (endogenous) oxidative DNA lesions play a role in this CS-specific premature-aging feature and supports the oxidative DNA damage theory of aging.
机译： CSB 基因的突变会导致库卡因综合症（CS），这是一种以紫外线敏感性以及严重的身体和神经功能受损为特征的DNA修复疾病。 CSB在核苷酸切除修复的转录偶联修复子途径中发挥作用。此功能可能解释了紫外线敏感性，但几乎无法阐明其他CS症状。其中许多，包括视网膜病变，都与早衰有关。我们在CS的小鼠模型中研究了眼病理。 Csb m / m 小鼠对紫外线非常敏感，并在角膜上皮上皮增生和鳞状细胞癌，这强调了转录的重要性-修复小鼠中的光损伤。此外，我们观察到 Csb m / m 视网膜中视网膜感光细胞的衰老随年龄的增长而自发减少，导致视网膜的感光细胞减少60％。 18个月大时的杆数。重要的是，当 Csb m / m 小鼠（以及 Csa ？/？小鼠）暴露于10 Gy的电离辐射中，我们注意到凋亡的感光细胞增加，这在野生型动物中未观察到。这项发现与我们观察到的建立的氧化应激标记基因的表达在 Csb m / m 视网膜中的表达上调有关，表明（内源性）氧化DNA损伤在CS特有的过早衰老特征中起作用，并支持氧化DNA损伤衰老理论。

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