als

Dag Stenberg stenberg at cc.Helsinki.FI
Sat Jul 16 13:12:08 EST 1994


Eliot Gelwan (eliot.gelwan at channel1.com) wrote:
> This is a consequence of the fact that the disease is one of the motor 
> neurons in the spinal cord as you suggest. 

You might have mentioned that ALS is a disease of BOTH the upper AND the
lower motoneuron; the upper motoneuron lesions lead to spasticity etc.
through still functioning motor units, and the lower motoneuron lesions 
progressively eliminate functioning motor units and lead to atrophy 
of the muscle etc.

An interesting goal for researchers will be to find out why the disease
affects motoneurons only, but both upper and lower ones. Understanding
the intracellular targeting mechanism of the disease might help to 
target therapy. 

The linkage to the SOD gene is in dispute, and anyway concerns only the
5% of patients expressing the hereditary form; hence considerably more
research is needed.

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