Retinal remodeling in the Tg P347L rabbit, a large-eye model of retinal degeneration.
Retinal remodeling in the Tg P347L rabbit, a large-eye model of retinal degeneration.
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DOI：
10.1002/cne.22703
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发表时间：
2011-10-01
影响因子：
2.5
通讯作者：
Marc, Robert Edward
中科院分区：
文献类型：
--
作者：
Jones, Bryan William;Kondo, Mineo;Terasaki, Hiroko;Watt, Carl Brock;Rapp, Kevin;Anderson, James;Lin, Yanhua;Shaw, Marguerite Victoria;Yang, Jia-Hui;Marc, Robert Edward
关键词：
视网膜色素变性（RP）是一种遗传性致盲疾病，其特征是视网膜光感受器的进行性丧失。有许多视网膜变性的啮齿动物模型，但大多数都是将转化为临床实践的干预措施的不良平台。兔具有许多视网膜疾病模型所需的品质，包括大眼睛和视网膜回路、解剖学和眼科学方面的现有和大量知识基础。我们已经分析了视网膜变性的兔模型中的变性、重塑和重编程，在TgP347L兔中表达视紫红质脯氨酸347至亮氨酸转基因作为研究视网膜变性的病理生理学和治疗的有力模型。我们发现，TgP347L兔的疾病进展密切跟踪人类保留视锥的RP，包括视锥相关的双极细胞信号转导和触发重编程的保存。相对较快的疾病进展使得TgP347L兔成为基因治疗、细胞生物学干预、祖细胞移植、外科干预和仿生假体研究的极好模型。
Retinitis pigmentosa (RP) is an inherited blinding disease characterized by progressive loss of retinal photo-receptors. There are numerous rodent models of retinal degeneration, but most are poor platforms for interventions that will translate into clinical practice. The rabbit possesses a number of desirable qualities for a model of retinal disease including a large eye and an existing and substantial knowledge base in retinal circuitry, anatomy, and ophthalmology. We have analyzed degeneration, remodeling, and reprogramming in a rabbit model of retinal degeneration, expressing a rhodopsin proline 347 to leucine transgene in a TgP347L rabbit as a powerful model to study the pathophysiology and treatment of retinal degeneration. We show that disease progression in the TgP347L rabbit closely tracks human cone-sparing RP, including the cone-associated preservation of bipolar cell signaling and triggering of reprogramming. The relatively fast disease progression makes the TgP347L rabbit an excellent model for gene therapy, cell biological intervention, progenitor cell transplantation, surgical interventions, and bionic prosthetic studies.
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影响因子：
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