• Clinical themes
• 33 min
  1. The clinical features of amyotrophic lateral sclerosis: diagnosis, natural history and epidemiology

  • Prof. Kevin Talbot
• 19 min
  2. Mechanisms of pathogenesis and molecular targets in spinal and bulbar muscular atrophy

  • Dr. Carlo Rinaldi
• Scientific themes
• 39 min
  3. Neuroinflammation in ALS: cause or consequence?

  • Prof. Philip Van Damme
• 44 min
  4. Cognition in ALS and the overlap with frontotemporal dementia (FTD)

  • Dr. Thomas Bak
• 39 min
  5. Large scale genetics of neurodegenerative diseases and ALS

  • Dr. Luc Dupuis
• 26 min
  6. SOD1-related ALS: what has it told us about motor neuron degeneration? - part 1

  • Prof. Dame Pamela Shaw
• 34 min
  7. SOD1-related ALS: what has it told us about motor neuron degeneration? - part 2

  • Prof. Dame Pamela Shaw
• 47 min
  8. Expanding roles of RNA-binding proteins in neurodegenerative diseases

  • Prof. Aaron D. Gitler
• 33 min
  9. Oxidative stress in amyotrophic lateral sclerosis (ALS) 1

  • Prof. Dame Pamela Shaw
• 20 min
  10. Oxidative stress in amyotrophic lateral sclerosis (ALS) 2

  • Prof. Dame Pamela Shaw
• 35 min
  11. Mechanisms of juvenile forms of ALS caused by FUS mutations

  • Dr. Luc Dupuis
• 21 min
  12. Importance of nutrition and weight loss in motor neuron disease

  • Dr. Luc Dupuis

Printable Handouts

PDF

Navigable Slide Index

1. Introduction
2. Plan (5)
3. Oxidative stress in human ALS
4. Evidence that oxidative stress plays a role in non-SOD1 MND
5. CSF 3-nitrotyrosine levels
6. Oxidative stress induces TDP-43 proteinopathic changes
7. Chronic oxidative stress induces wild type TDP-43 protein aggregates
8. Oxidative stress in C9ORF72 molecular pathophysiology
9. Oxidative stress at the neuromuscular junction
10. Plan (6)
11. 11 main pathophysiological mechanisms in ALS/MND
12. Oxidative stress is a central mechanism in ALS/MND
13. Links between glutamate and oxidative stress
14. Oxidative stress in MND pathophysiology
15. Targets damaged by ROS
16. Antioxidant capacity declines and oxidative stress increases with age
17. Plan (7)
18. Potential mechanisms of action of anti-oxidant drugs
19. Antioxidant treatment for ALS/MND Cochrane Database Systematic Review 2007
20. Clinical trials of anti-oxidant therapies in ALS/MND (1)
21. Clinical trials of anti-oxidant therapies in ALS/MND (2)
22. Edaravone trials: key results
23. Phase I copper ATSM trial
24. Potential of Nrf2-ARE activators for ALS/MND
25. Nuclear factor (erythroid-derived 2)-like 2: ‘NRF2’
26. Conclusions
27. Acknowledgments: research funding agencies

Topics Covered

• Evidence for oxidative stress in other subtypes of Motor Neuron Disease (MND)
• Causes of oxidative stress in MND
• The role of TDP-43 in MND
• Eleven main pathophysiological mechanisms in ALS/MND
• Oxidative stress as a therapeutic target in MND
• Ebselen
• Riluzole
• Edaravone, copper ATSM, and Dimethyl fumarate trials

Links

Series:

• ALS and Other Motor Neuron Disorders

Categories:

• Immunology
• Physiology & Anatomy

Therapeutic Areas:

• Immunology & Inflammation

External Links

• Slide 23: Copper ATSM clinical trial

Talk Citation

Publication History

• Published on September 29, 2022

Financial Disclosures

• Prof. Dame Pamela Shaw has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.