Autophagy in astrocytes: A novel culprit in lysosomal storage disorders

Chiara Di Malta, John D. Fryer, Carmine Settembre, Andrea Ballabio

Research output: Contribution to journalShort survey

22 Scopus citations

Abstract

Neurodegeneration is a prominent feature of lysosomal storage disorders (LSDs). Emerging data identify autophagy dysfunction in neurons as a major component of the phenotype. However, the autophagy pathway in the CNS has been studied predominantly in neurons, whereas in other cell types it has been largely unexplored. We studied the lysosome-autophagic pathway in astrocytes from a murine model of multiple sulfatase deficiency (MSD), a severe form of LSD. Similar to what was observed in neurons, we found that lysosomal storage in astrocytes impairs autophagosome maturation and this, in turn, has an impact upon the survival of cortical neurons and accounts for some of the neurological features found in MSD. Thus, our data indicate that lysosomal/ autophagic dysfunction in astrocytes is an important component of neurodegeneration in LSDs.

Original languageEnglish (US)
Pages (from-to)1871-1872
Number of pages2
JournalAutophagy
Volume8
Issue number12
DOIs
StatePublished - Dec 2012

Keywords

  • Astrocyte
  • Autophagy
  • Lysosomal storage disorders
  • Lysosome
  • Neurodegeneration

ASJC Scopus subject areas

  • Molecular Biology
  • Cell Biology

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