A Comprehensive Study of the Retinal Phenotype of Rpe65-Deficient Dogs.
A Comprehensive Study of the Retinal Phenotype of Rpe65-Deficient Dogs.
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DOI：
10.3390/cells10010115
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发表时间：
2021-01-09
期刊：
影响因子：
6
通讯作者：
Petersen-Jones SM
中科院分区：
文献类型：
--
作者：
Annear MJ;Mowat FM;Occelli LM;Smith AJ;Curran PG;Bainbridge JW;Ali RR;Petersen-Jones SM
Rpe65 缺陷狗对于 Leber 先天性黑蒙 2 型 (LCA2) 转化疗法的开发非常重要。本研究的目的是提供该狗模型视网膜变化自然史的综合报告。通过眼底成像、视网膜电图 (ERG) 和视力测试 (VT) 对 2 个月至 10 岁的 Rpe65 缺陷狗进行评估。通过光学相干断层扫描和塑料视网膜切片评估视网膜层厚度的变化。 ERG 显示视网膜敏感性明显丧失，幅度随着年龄的增长而下降。视网膜变薄最初发生在中央区域，其他区域的外视网膜变薄较慢，从下视网膜开始。 VT 表明，所有年龄段的狗在明亮的光线下都表现良好，而在较低的光线水平下它们会失明。视网膜色素上皮（RPE）内含物在较年轻的狗中发育，并且随着年龄的增长而增大。 ERG 振幅的下降反映了光感受器的丧失。缓慢的退化意味着年老的狗仍然有足够的光感受器，尽管非常不敏感，但仍能保持残余的亮光视觉。这项研究展示了 Rpe65 缺陷狗 LCA2 模型的自然史。
The Rpe65-deficient dog has been important for development of translational therapies of Leber congenital amaurosis type 2 (LCA2). The purpose of this study was to provide a comprehensive report of the natural history of retinal changes in this dog model. Rpe65-deficient dogs from 2 months to 10 years of age were assessed by fundus imaging, electroretinography (ERG) and vision testing (VT). Changes in retinal layer thickness were assessed by optical coherence tomography and on plastic retinal sections. ERG showed marked loss of retinal sensitivity, with amplitudes declining with age. Retinal thinning initially developed in the area centralis, with a slower thinning of the outer retina in other areas starting with the inferior retina. VT showed that dogs of all ages performed well in bright light, while at lower light levels they were blind. Retinal pigment epithelial (RPE) inclusions developed and in younger dogs and increased in size with age. The loss of photoreceptors was mirrored by a decline in ERG amplitudes. The slow degeneration meant that sufficient photoreceptors, albeit very desensitized, remained to allow for residual bright light vision in older dogs. This study shows the natural history of the Rpe65-deficient dog model of LCA2.
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影响因子：
4.2
作者：
Cideciyan, Artur V.;Hauswirth, William W.;Jacobson, Samuel G.
通讯作者：
Jacobson, Samuel G.
RPE65的慢病毒基因转移在Leber先天性amurosis小鼠模型中挽救了锥体的生存和功能。
影响因子：
15.8
作者：
Bemelmans, Alexis-Pierre;Kostic, Corinne;Crippa, Sylvain V.;Hauswirth, William W.;Lem, Janis;Munier, Francis L.;Seeliger, Mathias W.;Wenzel, Andreas;Arsenijevic, Yvan
通讯作者：
Arsenijevic, Yvan
影响因子：
4.4
作者：
Chen, Y;Moiseyev, Q;Ma, JX
通讯作者：
Ma, JX
影响因子：
4.2
作者：
Hauswirth, William W.;Aleman, Tomas S.;Jacobson, Samuel G.
通讯作者：
Jacobson, Samuel G.
影响因子：
12.4
作者：
Dejneka, NS;Surace, EM;Bennett, J
通讯作者：
Bennett, J