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Motor neuron disease

The curious ways of ALS

Nature volume 454, pages 284–285 (2008)Cite this article

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. Reporting in Genes & Development, Nishitoh et al.1 provide new insight into the molecular events that provoke ALS, linking its pathogenesis to a well-established stress pathway in an intracellular organelle called the endoplasmic reticulum.

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Figure 1: Proposed mechanisms of toxicity in SOD1-mediated ALS.

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Authors and Affiliations

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

    Magdalini Polymenidou & Don W. Cleveland

Authors
  1. Magdalini Polymenidou
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  2. Don W. Cleveland
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Polymenidou, M., Cleveland, D. The curious ways of ALS. Nature 454, 284–285 (2008). https://doi.org/10.1038/454284a

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