Bascom Palmer Eye Institute, Miller School of Medicine, University of Miami, Miami, FL 33136, USA.
Adv Exp Med Biol. 2012;723:93-9. doi: 10.1007/978-1-4614-0631-0_13.
Secondary cone degeneration in the transgenic rats carrying the S334ter rhodopsin mutation (S334ter-3 rats) starts at the peak of rod degeneration (PD12) and progresses with age. An early sign of cone degeneration is the loss of cone outer segments (COS) distributed in many small patches throughout the retina. Cone cell death occurs about 2 months later. When treated with CNTF (ciliary neurotrophic factor), impaired cones regenerate COS. Sustained delivery of CNTF prevents cones from degeneration and helps to maintain COS and function. These results indicate that cone degeneration is reversible at early stages, and supports a therapeutic strategy of sustained delivery of CNTF to prevent cone degeneration.
携带 S334ter 视蛋白突变的转基因大鼠（S334ter-3 大鼠）中的次级视锥细胞变性始于视杆细胞变性高峰期（PD12），并随年龄增长而进展。视锥细胞变性的早期迹象是分布在整个视网膜许多小斑块中的视锥细胞外节（COS）丢失。大约 2 个月后，视锥细胞死亡。用睫状神经营养因子（CNTF）治疗时，受损的视锥细胞再生 COS。CNTF 的持续递送可防止视锥细胞变性，并有助于维持 COS 和功能。这些结果表明，在早期阶段，视锥细胞变性是可逆的，支持持续递送 CNTF 以预防视锥细胞变性的治疗策略。
