Song De-Juan, Bao Xiao-Li, Fan Bin, Li Guang-Yu
Department of Ophthalmology, The Second Hospital of Jilin University, Changchun, 130000, China.
Cell Mol Neurobiol. 2023 Apr;43(3):1037-1048. doi: 10.1007/s10571-022-01243-2. Epub 2022 Jul 6.
Retinitis pigmentosa (RP) is a group of genetic disorders resulting in inherited blindness due to the degeneration of rod and cone photoreceptors. The various mechanisms underlying rod degeneration primarily rely on genetic mutations, leading to night blindness initially. Cones gradually degenerate after rods are almost eliminated, resulting in varying degrees of visual disability and blindness. The mechanism of cone degeneration remains unclear. An understanding of the mechanisms underlying cone degeneration in RP, a highly heterogeneous disease, is essential to develop novel treatments of RP. Herein, we review recent advancements in the five hypotheses of cone degeneration, including oxidative stress, trophic factors, metabolic stress, light damage, and inflammation activation. We also discuss the connection among these theories to provide a better understanding of secondary cone degeneration in RP. Five current mechanisms of cone degenerations in RP Interactions among different pathways are involved in RP.
视网膜色素变性(RP)是一组遗传性疾病,由于视杆和视锥光感受器的退化导致遗传性失明。视杆细胞退化的各种潜在机制主要依赖基因突变,最初导致夜盲。视杆细胞几乎消除后,视锥细胞逐渐退化,导致不同程度的视力残疾和失明。视锥细胞退化的机制尚不清楚。了解RP(一种高度异质性疾病)中视锥细胞退化的潜在机制对于开发RP的新疗法至关重要。在此,我们综述了视锥细胞退化的五个假说的最新进展,包括氧化应激、营养因子、代谢应激、光损伤和炎症激活。我们还讨论了这些理论之间的联系,以更好地理解RP中的继发性视锥细胞退化。RP中视锥细胞退化的五种当前机制不同途径之间的相互作用参与了RP。