Autophagy dysregulation in amyotrophic lateral sclerosis

Sheng Chen, Xiaojie Zhang, Lin Song, Weidong Le

Research output: Contribution to journalArticlepeer-review

134 Scopus citations


Autophagy is an intracellular lysosomal degradation process, which plays an important role in cell growth and development, and keeping cellular homeostasis in all eukaryotes. Autophagy has multiple physiological functions, including protein degradation, organelle turnover and response to stress. Emerging evidences support the notion that dysregulation of autophagy might be critical for pathogenesis of amyotrophic lateral sclerosis (ALS). The autophagy dysregulation in motor neurons of ALS may occur in different steps of the autophagic process. Recent studies have shown that two ALS associated proteins, TDP-43 and superoxide dismutase 1 (SOD1), are involved in the abnormal autophagy regulation. Furthermore, it is reported that several genetic mutations in ALS disturb the autophagic process in the motor neurons. This review will provide new evidence of autophagy dysregulation as a critical pathogenic process leading to ALS, and will discuss the prospect of future therapeutic targets using autophagic regulation to treat this disease.

Original languageEnglish (US)
Pages (from-to)110-116
Number of pages7
JournalBrain Pathology
Issue number1
StatePublished - Jan 2012


  • amyotrophic lateral sclerosis
  • autophagy
  • Cu/Zn superoxide dismutase 1
  • dynein
  • dysregulation
  • lysosome

ASJC Scopus subject areas

  • Neuroscience(all)
  • Pathology and Forensic Medicine
  • Clinical Neurology


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