an ALS Hypothesis
kpaulc at earthlink.net
Mon Jan 31 21:02:26 EST 2000
must be an 'echo' in-here :-)
k. p. collins
dag.stenberg at helsinki.nospam.fi wrote:
> John H. <johnhkm at netsprintXXXX.net.au> wrote:
> > I thought it may have been a SOD problem, but haven't managed to have a
> > close look at it yet. Isn't there a familial tendency here also?
> > Would someone more knowledgeable than the current authors in this thread
> > care to enlighten? I know something about apoptosis etc but ALS .... .
> Fast attempt at partial answer:
> - ALS = amyotrophic lateral sclerosis in a degenreative disease
> afflicting both upper (brain, especially cortex) and lower (spinal cord
> plus lower brain stem) motor neurons selectively.
> - It leads to progressive paralysis and muscle atrophy because of the
> degeneration of lower motoneurons, and to spasticity due to the
> degeneration of upper motor neurons.
> - a synonym is Lou Gehrig's disease. A famous living patient is Steven
> Hawkings, a most famous scientist and public figure.
> - paralysis and atrophy usually start in limbs, not always symmetrically
> - the last to be afflicted is brain stem: swallowing, respiration etc.
> - sometimes concomitant symptoms, not clearly motor, show as emotional
> instability, proneness to laughter, crying etc.
> - the disease may start between 30 and 60 years of age
> - it progresses in some cases until death (from respiratory paralysis)
> but often stops from unknown reason, perhaps only paralyzing the legs,
> making the patient wheel-chair-bound.
> - no treatment yet, only symptomatic help
> This about the clinical picture.
> - in about 5-10% of patients there seems to be a familial predisposition
> - in these there has been shown mutations in the SOD1 gene
> - the article KPC wrote about is about a mouse model of lower motor
> neuron degeneration
> - in this article mitochondrial involvement is shown
> -- I hope this is all right, have to run....
> -- John, I have stored your question about the Lanfumey paper on 5HT1A.
> I will get back to it, but a certain urgent project is in its final
> Dag Stenberg
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