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Lab, Field, and Clinic
October 12, 2011

New Model System Offers Clues to ALS

JAMA. 2011;306(14):1534. doi:10.1001/jama.2011.1439

Using tissue samples collected from patients with amyotrophic lateral sclerosis (ALS), scientists have created a new in vitro model for the disease that is providing insights into the mechanism of the disorder.

ALS is a progressive and ultimately fatal disorder in which the motor neurons in a patient's central nervous system die off, causing paralysis and eventually respiratory failure. Most cases are sporadic, but about 20% are linked to mutations in the superoxide dismutase 1 (SOD1) gene. However, scientists have so far been unable to identify the precise mechanisms behind the motor neuron loss.

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