Artikel
Dissecting the role of lysosome dysfunction in AMD
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Veröffentlicht: | 5. Februar 2020 |
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Gliederung
Text
Age-related macular degeneration (AMD) is a neurodegenerative retinal disease and the leading cause of blindness in developed countries. AMD pathogenesis originates from the damage of a polarized non-regenerative retinal pigment epithelium (RPE) cells. The RPE performs many functions indispensable for vison, such as phagocytosing and degrading shed photoreceptor outer segments (POS). Degradation and recycling of cellular components are the main responsibilities attributed to lysosomes. Here we report the characterization of two RPE models featuring lysosomal dysfunction: a) chloroquine-induced in hPSc-RPE and b) POS-induced primary human fetal RPE (hfRPE) cells. These models are being used to explore the importance of lysosomes in intracellular cargo processing and the role of lysosomal dysfunction in AMD. Ultimately, the identification and characterization of defective pathways responsible for the regulation of lysosomal biogenesis and activity will contribute to a better understanding of AMD pathogenesis and identification of new therapeutic targets.