Retinal remodeling in inherited photoreceptor degenerations
Retinal remodeling in inherited photoreceptor degenerations
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DOI：
10.1385/mn:28:2:139
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发表时间：
2003-10-01
影响因子：
5.1
通讯作者：
Jones, BW
中科院分区：
文献类型：
--
作者：
Marc, RE;Jones, BW
视杆细胞或视网膜色素上皮细胞发生的光感受器变性通常引起继发性视锥细胞死亡和残存神经视网膜的感觉传入阻滞。在成熟的中枢神经系统中，传入神经阻滞引起萎缩和连接重建。人们一直认为，神经视网膜不会重塑，它是一个被动的幸存者。利用计算分子表型对人类和啮齿类动物视网膜变性晚期进行筛查，发现长期存在侵袭性负性重塑，其中神经元沿沿着异常胶质柱和密封迁移，重建成人神经视网膜（1）。许多神经元死亡，但幸存者重新连接残余的内丛状层（IPL），在残余的内核层（INL）形成数千个新的异位微神经瘤。双极细胞和无长突细胞参与新的回路，这些回路最有可能是腐败的。无论最初引发视网膜变性的分子缺陷如何，人类和啮齿动物视网膜的重塑都会出现。虽然重塑可能会限制治疗间隔的分子，细胞或仿生救援，暴露内在的视网膜重塑的感觉控制视网膜变性的去除表明，在正常视网膜的神经组织可能比以前认为的更具可塑性。
Photoreceptor degenerations initiated in rods or the retinal pigmented epithelium usually evoke secondary cone death and sensory deafferentation of the surviving neural retina. In the mature central nervous system, deafferentation evokes atrophy and connective re-patterning. It has been assumed that the neural retina does not remodel, and that it is a passive survivor. Screening of advanced stages of human and rodent retinal degenerations with computational molecular phenotyping has exposed a prolonged period of aggressive negative remodeling in which neurons migrate along aberrant glial columns and seals, restructuring the adult neural retina (1). Many neurons die, but survivors rewire the remnant inner plexiform layer (IPL), forming thousands of novel ectopic microneuromas in the remnant inner nuclear layer (INL). Bipolar and amacrine cells engage in new circuits that are most likely corruptive. Remodeling in human and rodent retinas emerges regardless of the molecular defects that initially trigger retinal degenerations. Although remodeling may constrain therapeutic intervals for molecular, cellular, or bionic rescue, the exposure of intrinsic retinal remodeling by the removal of sensory control in retinal degenerations suggests that neuronal organization in the normal retina may be more plastic than previously believed.