McMaster University

Demystifying Medicine
Seminar Series

ALS: The cell under stress

Please complete our feedback form

This video details the role of stress granules in the pathogenesis of Amyotrophic Lateral Sclerosis (ALS). It briefly describes ALS before delving into what makes stress granules so problematic for motor neurons. Having highlighted the key differences in a healthy vs. problematic cell, the video concludes by discussing some novel therapies on the market that target the stress granule pathway.

— Video by Prabhnoor Bhons, Mahrukh Aziz, Ashley Dhaliwal and Victoria Bednarek

References

  • Li Y, King O, Shorter J, Gitler A. Stress granules as crucibles of ALS pathogenesis. The Journal of Cell Biology. 2013Apr29;
  • Vance C, Scotter EL, Nishimura AL, Troakes C, Mitchell JC, Kathe C, et al. ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules. Human Molecular Genetics. 2013Jul;22(13):2676–88.
  • Marrone, L., Poser, I., Casci, I., Japtok, J., Reinhardt, P., Janosch, A., ... & Reinhardt, L (2018). Isogenic FUS-eGFP iPSC reporter lines enable quantification of FUS stress granule pathology that is rescued by drugs inducing autophagy. Stem Cell Reports, 10(2), 375-389.
  • Bosco DA, Lemay N, Ko HK, Zhou H, Burke C, Kwiatkowski Jr TJ, Sapp P, McKenna-Yasek D, Brown Jr RH, Hayward LJ. Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules. Human Molecular Genetics. 2010 Aug 10;19(21):4160-75.
  • Robberecht W, Philips T. The changing scene of amyotrophic lateral sclerosis. Nature Reviews Neuroscience. 2013 Apr;14(4):248.
  • Rowland LP, Shneider NA. Amyotrophic lateral sclerosis. New England Journal of Medicine. 2001 May 31;344(22):1688-700.
  • Milisav I. Cellular stress responses. In Advances in Regenerative Medicine 2011.
  • Statistics Canada. Neurological conditions in household population. Government of Canada. Retrieved Nov 18, 2018.
  • ALS Society of Canada. What is ALS? Retrieved Nov 18, 2018.