首页> 外文期刊>Investigative ophthalmology & visual science >Divergent Phenotypes of Vision and Accessory Visual Function in Mice with Visual Cycle Dysfunction (Rpe65rd12) or Retinal Degeneration (rd/rd).
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Divergent Phenotypes of Vision and Accessory Visual Function in Mice with Visual Cycle Dysfunction (Rpe65rd12) or Retinal Degeneration (rd/rd).

机译:患有视觉循环功能障碍(Rpe65rd12)或视网膜变性(rd / rd)的小鼠的视觉和辅助视觉功能有不同的表型。

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PURPOSE: Tests of vision for mice remain limited and the visual phenotype of some retinal disorders in mice remain poorly understood. A novel assay of vision was used to determine how the form and extent of retinal disease affects visual phenotype in mice. METHODS: Retinal histology, the suppression of locomotion by light and visual guidance of locomotion, were assessed in mice with progressive photoreceptor degeneration (rd/rd) or visual cycle dysfunction (Rpe65(rd12)). RESULTS: In wild-type mice, there was visual guidance of locomotor activity in dim light and suppression of activity (negative masking) in bright light. In rd/rd mice, vision was sufficient to guide locomotion at postnatal day (P)34 but was lost from P46 onward. In bright light rd/rd mice had enhanced negative masking. Although Rpe65(rd12) mice had no dim light response, with high illumination, vision was sufficient to guide locomotion at all ages tested. CONCLUSIONS: A major concern for gene and cell replacement therapies is the development of visual pathways through which restored retinal function can connect to visual centers of the brain. The residual retinal response to high illumination in Rpe65(rd12) mice translates into useful vision, and visual pathways remain functional-a prerequisite for restoring vision in disorders of the retina. Similarly, useful vision in young rd/rd mice shows that there is visual pathway function before photoreceptor degeneration and suggests the potential for early therapy. Together, these findings recommend observation of masking responses in the assessment of gene and cell replacement therapies.
机译:目的:对小鼠的视力测试仍然有限,并且对小鼠某些视网膜疾病的视觉表型仍然知之甚少。一种新颖的视力测定法用于确定视网膜疾病的形式和程度如何影响小鼠的视觉表型。方法:在进行性光感受器变性(rd / rd)或视觉周期功能障碍(Rpe65(rd12))的小鼠中评估视网膜组织学,光对运动的抑制和视觉对运动的指导。结果:在野生型小鼠中,在昏暗的灯光下有视觉上的运动活动指导,在明亮的灯光下有视觉上的活动抑制作用(负掩蔽)。在rd / rd小鼠中,视力足以指导产后一天(P)34的运动,但从P46开始失去了视力。在明亮的灯光下,rd / rd小鼠的负掩蔽性增强。尽管Rpe65(rd12)小鼠没有昏暗的灯光反应,但在高光照下,视力足以指导所有测试年龄的运动。结论:基因和细胞替代疗法的主要关注是视觉通路的发展,通过该通路,视网膜功能恢复可以连接到大脑的视觉中心。 Rpe65(rd12)小鼠在高照度下的残余视网膜反应转化为有用的视力,而视力通路仍保持功能性-在视网膜疾病中恢复视力的前提。同样,在年轻的rd / rd小鼠中有用的视力表明,在感光器退化之前存在视觉通路功能,并暗示了早期治疗的潜力。总之,这些发现建议在评估基因和细胞替代疗法时观察掩盖反应。

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