Telethon Institute of Genetics and Medicine (TIGEM), Via Pietro Castellino 111, 80131, Naples, Italy.
Nat Rev Mol Cell Biol. 2013 May;14(5):283-96. doi: 10.1038/nrm3565.
For a long time, lysosomes were considered merely to be cellular 'incinerators' involved in the degradation and recycling of cellular waste. However, now there is compelling evidence indicating that lysosomes have a much broader function and that they are involved in fundamental processes such as secretion, plasma membrane repair, signalling and energy metabolism. Furthermore, the essential role of lysosomes in autophagic pathways puts these organelles at the crossroads of several cellular processes, with significant implications for health and disease. The identification of a master regulator, transcription factor EB (TFEB), that regulates lysosomal biogenesis and autophagy has revealed how the lysosome adapts to environmental cues, such as starvation, and targeting TFEB may provide a novel therapeutic strategy for modulating lysosomal function in human disease.
长期以来,溶酶体被认为仅仅是参与细胞废物降解和再循环的细胞“焚化炉”。然而,现在有令人信服的证据表明,溶酶体具有更广泛的功能,它们参与诸如分泌、质膜修复、信号转导和能量代谢等基本过程。此外,溶酶体在自噬途径中的重要作用使这些细胞器处于几个细胞过程的交汇点,对健康和疾病有重大影响。鉴定出一种主调控因子,即转录因子 EB(TFEB),它调节溶酶体生物发生和自噬,揭示了溶酶体如何适应饥饿等环境信号,而靶向 TFEB 可能为调节人类疾病中的溶酶体功能提供一种新的治疗策略。