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Oxidative stress in amyotrophic lateral sclerosis (ALS) 1
Published on September 29, 2022 33 min
Other Talks in the Series: ALS and Other Motor Neuron Disorders
Cognition in ALS and the overlap with frontotemporal dementia (FTD)
- Dr. Thomas Bak
- University of Edinburgh, UK
My name is Professor Dame Pamela Shaw. I'm from the Neurology Department at the University of Sheffield and the Sheffield Institute for Translational Neuroscience. The topic of my lecture is Oxidative Stress in Amyotrophic Lateral Sclerosis (ALS).
I have no disclosures relevant to this lecture.
What I'm going to cover during the lecture is on these seven areas. First of all, I'm going to introduce you to Motor Neuron Disease or ALS, then I will cover the mechanisms of neuronal oxidative stress, and then discuss antioxidant defence mechanisms. I will then go over the evidence for the role of oxidative stress in SOD1-related MND. Then cover the evidence for oxidative stress in other sub-types of this disease. I'll then review the causes of oxidative stress in MND, and in the final section talk about oxidative stress as a potential therapeutic target.
ALS or Motor Neuron Disease was first described by the French physician, Jean-Martin Charcot, at the Salpetriere Hospital, in Paris in 1869.
What motor neurons do, in a nutshell, is form a communication between the brain and the muscle. We have this beautiful network of processes or axons from motor neurons that come out from the central nervous system into the peripheral nerves and form a contact eventually with muscle fibres. Motor neurons are the largest cells in the body. They may have an axon up to a metre in length, if you think about a motor neuron in the lower spinal cord connecting with a muscle in the lower part of the leg, and so they're very large, specialised cells with very long processes. If you imagine the size of the cell body as being the size of a tennis ball, if you transpose it in your mind's eye, then the relevant axon would be up to 2 kilometres in length. Amazing cells with amazing properties.