Filtration of Short-Wavelength Light Provides Therapeutic Benefit in Retinitis Pigmentosa Caused by a Common Rhodopsin Mutation.
Filtration of Short-Wavelength Light Provides Therapeutic Benefit in Retinitis Pigmentosa Caused by a Common Rhodopsin Mutation.
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短波长光的过滤可为常见视紫红质突变引起的色素性视网膜炎提供治疗效果。
DOI：
10.1167/iovs.19-26964
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发表时间：
2019
影响因子：
4.4
通讯作者：
Orlans HO
中科院分区：
文献类型：
--
作者：
Orlans HO
目的：光照在加速视网膜色素变性（RP）中的作用仍然存在争议。然而，在多种形式（“扇区”RP）中观察到下视网膜比上视网膜更快的变性，包括由视紫红质 P23H 突变引起的变性，表明在这种情况下入射光暴露的改变作用。在完全黑暗的环境中饲养同等的动物模型已被证明可以减缓退化。在这里，我们研究使用红色滤光片作为一种潜在的治疗策略，假设最大限度地减少视网膜暴露于视杆细胞最敏感的< 600 nm的光可能会提供治疗益处。方法：将饲养在红色塑料笼中的 P23H 显性视紫红质突变 (Rho P23H/+) 杂合的 Knockin 小鼠在断奶时分为无色或红色笼子。随后，通过谱域眼相干断层扫描测量光感受器层 (PRL) 厚度，通过 ERG 量化视网膜功能，并通过视网膜平片的免疫组织化学分析 (IHC) 确定视锥形态。结果：在所有时间点，留在红色笼子中的小鼠的 PRL 厚度均显着大于饲养在无色笼子中的小鼠。根据暗适应和光适应 ERG 反应，红色外壳还可以显着挽救视网膜功能。 IHC 进一步揭示了与透明饲养组相比，红色视锥细胞形态和数量的显着改善。结论：短波长光暴露的限制显着减缓了 Rho P23H/+ 小鼠模型的退化。对于患有扇形表型的视杆锥体营养不良患者来说，红色滤光片可能代表一种经济有效且低风险的治疗方法。
Purpose: The role of light exposure in accelerating retinitis pigmentosa (RP) remains controversial. Faster degeneration has however been observed in the inferior than superior retina in several forms (“sector” RP), including those caused by the rhodopsin P23H mutation, suggesting a modifying role of incident light exposure in such cases. Rearing of equivalent animal models in complete darkness has been shown to slow the degeneration. Here we investigate the use of red filters as a potential treatment strategy, with the hypothesis that minimizing retinal exposure to light< 600 nm to which rods are maximally sensitive may provide therapeutic benefit.Methods: Knockin mice heterozygous for the P23H dominant rhodopsin mutation (Rho P23H/+) housed in red-tinted plastic cages were divided at weaning into either untinted or red-tinted cages. Subsequently, photoreceptor layer (PRL) thickness was measured by spectral-domain ocular coherence tomography, retinal function quantified by ERG, and cone morphology determined by immunohistochemical analysis (IHC) of retinal flatmounts.Results: Mice remaining in red-tinted cages had a significantly greater PRL thickness than those housed in untinted cages at all time points. Red housing also led to a highly significant rescue of retinal function as determined by both dark-and light-adapted ERG responses. IHC further revealed a dramatic benefit on cone morphology and number in the red-as compared with the clear-housed group.Conclusions: Limitation of short-wavelength light exposure significantly slows degeneration in the Rho P23H/+ mouse model. Red filters may represent a cost-effective and low-risk treatment for patients with rod-cone dystrophy in whom a sectoral phenotype is noted.
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作者：
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发表时间：
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表达突变视紫红质的转基因小鼠中光诱导的光感受器变性加速。
影响因子：
4.4
作者：
Naash,ML;Peachey,NS;Li,ZY;Gryczan,CC;Goto,Y;Blanks,J;Milam,AH;Ripps,H
通讯作者：
Ripps,H