Article
Mutant SOD1-expressing astrocytes release toxic factors that trigger motoneuron death by inducing hyperexcitability.
Journal of neurophysiology - 1 Jun 2013
Fritz Elsa, Izaurieta Pamela, Weiss Alexandra, Mir Franco R, Rojas Patricio, Gonzalez David, Rojas Fabiola, Brown Robert H, Madrid Rodolfo, van Zundert Brigitte
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a devastating paralytic disorder caused by dysfunction and degeneration of motoneurons starting in adulthood. Recent studies using cell or animal models document that astrocytes expressing disease-causing mutations of human superoxide dismutase 1 (hSOD1) contribute to the pathogenesis of ALS by releasing a neurotoxic factor(s). Neither the mechanism by which this neurotoxic...
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