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Nature Medicine 10, 905 - 906 (2004)
doi:10.1038/nm0904-905

Maiming mitochondria in familial ALS

Valina L Dawson1

  1. Valina L. Dawson is at the Institute for Cell Engineering and in the Departments of Neurology, Neuroscience and Physiology, John Hopkins University School of Medicine, Baltimore Maryland, 21205, USA. e-mail: vdawson@jhmi.edu


A population of neurons in patients with familial amyotrophic lateral sclerosis have multiple abnormalities, including protein aggregation and mitochondrial dysfunction. Two studies now reveal interactions between these two defects.


Something is not quite right about the mitochondria of patients with familial amyotrophic lateral sclerosis (ALS). Vacuolation and degeneration of mitochondria occur early in this degenerative disease, which afflicts 6 out of 100,000 individuals.

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