Could Sirtuin Activities Modify ALS Onset and Progression?

Cell Mol Neurobiol. 2017 Oct;37(7):1147-1160. doi: 10.1007/s10571-016-0452-2. Epub 2016 Dec 10.


Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with a complex etiology. Sirtuins have been implicated as disease-modifying factors in several neurological disorders, and in the past decade, attempts have been made to check if manipulating Sirtuin activities and levels could confer benefit in terms of neuroprotection and survival in ALS models. The efforts have largely focused on mutant SOD1, and while limited in scope, the results were largely positive. Here, the body of work linking Sirtuins with ALS is reviewed, with discussions on how Sirtuins and their activities may impact on the major etiological mechanisms of ALS. Moving forward, it is important that the potentially beneficial effect of Sirtuins in ALS disease onset and progression are assessed in ALS models with TDP-43, FUS, and C9orf72 mutations.

Keywords: Amyotrophic lateral sclerosis (ALS); Motor neuron; Neuronal degeneration; Sirtuins.

Publication types

  • Review

MeSH terms

  • Amyotrophic Lateral Sclerosis / drug therapy
  • Amyotrophic Lateral Sclerosis / metabolism*
  • Amyotrophic Lateral Sclerosis / pathology*
  • Animals
  • Disease Progression*
  • Humans
  • Motor Neurons / drug effects
  • Motor Neurons / metabolism
  • Motor Neurons / pathology
  • Sirtuins / metabolism*
  • Sirtuins / therapeutic use*
  • Superoxide Dismutase-1 / metabolism


  • Superoxide Dismutase-1
  • Sirtuins