自噬体和溶酶体是细胞维持稳态的重要系统,自噬体负责底物的识别和包裹,溶酶体负责底物的降解。溶酶体功能紊乱会导致细胞内物质不能被正常降解、致病性底物发生蓄积,进而诱发多种重大疾病,如溶酶体蓄积病(lysosomal storage disorders, LSDs)、神经退行性疾病和代谢性疾病等;相反,促进溶酶体生成,增强其降解功能则具有改善疾病的作用。因此,揭示并阐明溶酶体生成的调控机制是重要的科学问题。本文对溶酶体生成调控领域近年的研究进展进行综述。

Autophagosomes and lysosomes are crucial intracellular systems for the regulation of homeostasis. Autophagosomes are responsible for cargo recognition, while lysosomes are responsible for cargo degradation. Lysosomal dysfunction leads to defective degradation of intracellular cargos and accumulation of pathogenic aggregates, which subsequently promotes the pathogenesis of various disorders, including lysosomal storage disorders (LSDs), neurodegenerative diseases and metabolic diseases. Conversely, enhanced lysosome biogenesis and lysosomal function ameliorate human diseases. Therefore, it is an important scientific issue to reveal and elucidate the regulatory mechanisms of lysosome biogenesis. In this article, recent advances in the regulation of lysosome biogenesis are reviewed.