Neuroinflammation in ALS: cause or consequence?

Published on June 30, 2016   39 min
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Hello. I am Philip Van Damme from the University of Leuven Belgium. It is my pleasure to contribute to this lecture series about ALS, and I will talk about the role of neuroinflammation in ALS. The title of my talk is, "NeuroInflammation in ALS, Cause or Consequence?"
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The signs and symptoms of amyotrophic lateral sclerosis, or ALS are caused by the degeneration of upper motor neurons in the motor cortex and lower motor neurons in the brain stem and spinal cord. This degeneration of motor neurons is the hallmark of ALS, but it can manifest itself in various ways. ALS is a heterogeneous disorder with striking variability in clinical presentation, in the genetic causes, and in the underlying disease proteins. Already at the clinical level, ALS can come in different flavors. The side of onset is mostly focal width onset in limb muscles or bulbar muscles. The age at onset has a wide range, from around 18 years of age to over 85 years. The rate of disease progression can be fast or slow. And the extent of upper versus lower motor neuron involvement, and the extent of frontal temporal involvement varies from patient to patient. Also, the genetic causes that can be identified in about 10% of patients range from missense mutations in superoxide dismutase one, or SOD1, tar DNA-binding protein for T3, or TARDBP, and fused in sarcoma, or FUS. To repeat expansions of a G-4, C-2 hexanucleotide repeat expansion in the chromozome 9 open reading frame 72, or C9orf72 gene. The underlying disease protein is mostly TDP-43, but can also be FUS, SOD1, or dipeptide repeats in the different genetic subtypes of ALS.
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Neuroinflammation in ALS: cause or consequence?

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