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Nature 454, 284-285 (17 July 2008) | doi:10.1038/454284a; Published online 16 July 2008

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Motor neuron disease: The curious ways of ALS

Magdalini Polymenidou1 & Don W. Cleveland1

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That mutations in the SOD1 enzyme underlie inherited forms of a motor neuron disease known as ALS is clear. But the question of what the consequences of such mutations are seems to have more than one answer.

Amyotrophic lateral sclerosis (ALS) is a neurological disorder characterized by the selective premature degeneration and death of motor neurons, which control voluntary actions such as breathing and walking. The disease starts in adult life, and the ensuing progressive paralysis is typically fatal within a few years, usually owing to failure of the respiratory system.

  1. Magdalini Polymenidou and Don W. Cleveland are at the Ludwig Institute for Cancer Research, and the Department of Cellular and Molecular Medicine, University of California, San Diego, 9500 Gilman Drive, La Jolla, California 92093–00670, USA.
    Email: dcleveland@ucsd.edu

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