TRPML1-/TFEB-Dependent Regulation of Lysosomal Exocytosis

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

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.

Keywords: Autophagy; Calcineurin; LSDs; Lysosomal calcium; TFEB.

MeSH terms

  • Animals
  • Autophagy
  • Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / metabolism*
  • Calcium / metabolism
  • Exocytosis*
  • Humans
  • Lysosomal Storage Diseases / metabolism
  • Lysosomes / metabolism*
  • Signal Transduction*
  • Transient Receptor Potential Channels / metabolism*

Substances

  • Basic Helix-Loop-Helix Leucine Zipper Transcription Factors
  • MCOLN1 protein, human
  • TFEB protein, human
  • Transient Receptor Potential Channels
  • Calcium