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1 2005

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Progressive die-back and death of motoneurons in a transgenic mouse model of amyotrophic lateral sclerosis

Gordon T., Tam S. L., Sharp P. S., Hegedus. J.

Biocybernetics and Biomedical Engineering

2005

Vol. 25, no. 4

17-26

Fischer et al. [1] commented that the longest and largest nerve fibers with the highest metabolic demand appear to be the most susceptible to "dying-back" in a wide variety of degenerative and toxic conditions of the central and peripheral nervous systems. In the high copy number G93AmSOD1 transgenic mouse model of familial amyotrophic lateral sclerosis (ALS) we tested the hypothesis that the largest motoneurons which have the most terminal connections are the most susceptible to the disease. In a time course study of motoneuron, muscle and motor unit properties in fast - and slow-twitch hindlimb muscles of G93A and wild type mice, we found a rapid decline in numbers of functional motor units and motoneurons that progresses from birth to a plateau after 90-100 days of life with surprisingly Iittle compensatory axonal sprouting. Fast motoneurons are the most susceptible, contrasting with the slow motoneurons. Preliminary evidence of loss of S100 reactive perisynaptic Schwann cells at the denervated endplate regions of the affected muscles indicate loss paralIeIs rapid progression of disease with consequent decline in muscle force and motor unit numbers, folIowed closely in time by motoneuron death.