溶酶体胞吐作用的TRPML1- / TFEB依赖性调控。

TRPML1-/TFEB-Dependent Regulation of Lysosomal Exocytosis.

作者信息

Di Paola Simone, Medina Diego L

机构信息

Telethon Institute of Genetics and Medicine (TIGEM), Pozzuoli, NA, Italy.

出版信息

Methods Mol Biol. 2019;1925:143-144. doi: 10.1007/978-1-4939-9018-4_12.

DOI:10.1007/978-1-4939-9018-4_12

PMID:30674023

Abstract

Emerging experimental evidences indicate that the lysosome can trigger a calcium signaling, via TRPML1/calcineurin/TFEB pathway, that promotes lysosomal exocytosis and clearance of lysosomal accumulation in various cellular models of lysosomal storage disorders (LSDs). Here, we described methods to determine TFEB activation and lysosomal exocytosis that may represent innovative tools to study lysosomal function and to develop novel therapeutic approaches to promote clearance in LSDs.

摘要

新出现的实验证据表明，溶酶体可通过TRPML1/钙调神经磷酸酶/转录因子EB（TFEB）途径触发钙信号传导，在溶酶体贮积症（LSDs）的各种细胞模型中促进溶酶体胞吐作用和溶酶体蓄积物的清除。在此，我们描述了确定TFEB激活和溶酶体胞吐作用的方法，这些方法可能是研究溶酶体功能以及开发促进LSDs清除的新治疗方法的创新工具。