视网膜变性
自噬
色素性视网膜炎
细胞生物学
生物
下调和上调
视网膜
视网膜
自噬体
感光细胞
程序性细胞死亡
TFEB
外层核层
PI3K/AKT/mTOR通路
变性(医学)
神经科学
细胞内
黄斑变性
视网膜再生
溶酶体
视网膜电图
视网膜色素上皮
作者
Kirstan A. Vessey,Nadia Hosseini Naveh,Ophelia Ehrlich,A. S. B. Glover,J. J. Lee,Ursula Greferath,Andrew I. Jobling,Erica L. Fletcher
出处
期刊:Cells
[Multidisciplinary Digital Publishing Institute]
日期:2026-02-13
卷期号:15 (4): 345-345
标识
DOI:10.3390/cells15040345
摘要
Inherited retinal degenerations, such as retinitis pigmentosa, are a leading cause of irreversible vision loss, yet broadly effective treatments remain elusive. Impaired cellular waste clearance via autophagy-lysosomal pathways have been implicated in photoreceptor death, but the spatiotemporal dynamics of these processes during degeneration remain poorly understood. Using the rd10 mouse model of retinitis pigmentosa, we characterised autophagy-lysosomal dysfunction at key stages of photoreceptor degeneration (postnatal day P17, P22, P35) through super-resolution imaging of RFP-EGFP-LC3 reporter mice, Western blot, and bulk RNA sequencing. Autophagosome and autolysosome numbers were significantly elevated across all photoreceptor compartments (inner/outer segments, outer nuclear layer, outer plexiform layer) at P17, prior to significant photoreceptor nuclei loss. Autophagosome and autolysosome size progressively increased from P22 onwards, suggesting accumulation of unprocessed intracellular waste. Molecular analyses revealed downregulation of mTOR protein, upregulation of autophagy-related genes, and increased lysosomal processes from P17. These histological and molecular findings are consistent with early autophagy induction followed by overwhelmed degradative capacity. Our findings identify autophagy-lysosomal change as an early event in photoreceptor loss in the rd10 model, revealing a critical therapeutic window for mutation-independent interventions targeting cellular clearance pathways in inherited retinal degenerations.
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