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0:00
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).
0:21
I have no disclosures
relevant to this lecture.
0:27
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.
1:15
ALS or Motor Neuron Disease
was first described by
the French physician,
Jean-Martin Charcot, at
the Salpetriere Hospital,
in Paris in 1869.
1:29
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.
