Inhibition of Lysosome Membrane Recycling Causes Accumulation of Gangliosides that Contribute to Neurodegeneration

Maxime Boutry, Julien Branchu, Céline Lustremant, Claire Pujol, Julie Pernelle, Raphaël Matusiak, Alexandre Seyer, Marion Poirel, Emeline Chu-Van, Alexandre Pierga, Kostantin Dobrenis, Jean Philippe Puech, Catherine Caillaud, Alexandra Durr, Alexis Brice, Benoit Colsch, Fanny Mochel, Khalid Hamid El Hachimi, Giovanni Stevanin, Frédéric Darios

Research output: Contribution to journalArticlepeer-review

53 Scopus citations

Abstract

Boutry et al. show that inhibition of lysosome membrane recycling leads to lysosomal accumulation of some glycosphingolipids (simple gangliosides), which contributes to neuronal death.

Original languageEnglish (US)
Pages (from-to)3813-3826
Number of pages14
JournalCell Reports
Volume23
Issue number13
DOIs
StatePublished - Jun 26 2018

Keywords

  • autophagic lysosome recovery
  • autophagy
  • glycosphingolipids
  • induced pluripotent stem cells
  • knockout
  • lipid metabolism
  • lysosomes
  • membrane trafficking
  • neurodegenerative disease
  • organoids

ASJC Scopus subject areas

  • General Biochemistry, Genetics and Molecular Biology

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