Altered macroautophagy in the spinal cord of SOD1 mutant mice

Ang Li, Xiaojie Zhang, Weidong Le

Research output: Contribution to journalArticle

144 Scopus citations

Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease caused by selective loss of motor neurons (MNs). About 20% familial cases of ALS (fALS) carried the Cu, Zn-superoxide dismutase (SOD1) gene mutation, which plays a crucial role in the pathogenesis of fALS. There is evidence suggesting that macroautophagy can degrade mutated SOD1 in vitro. To investigate whether the mutant SOD1 can induce macroautophagy in vivo, we examined the LC3 processing in spinal cord and the activation status of macroautophagy in MNs of SOD1 G93A transgenic mice at different stages. Our data demonstrated that autophagy was activated in spinal cord of SOD1G93A mice indicating a possible role of macroautophagy in the pathogenesis of ALS.

Original languageEnglish (US)
Pages (from-to)290-293
Number of pages4
JournalAutophagy
Volume4
Issue number3
DOIs
StatePublished - Apr 1 2008

Keywords

  • Amyotrophic lateral sclerosis
  • Macroautophagy
  • Motor neurons
  • SOD1
  • Transgenic mice

ASJC Scopus subject areas

  • Cell Biology
  • Molecular Biology

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