Article
[ALS disease modeling and drug screening using patient-specific iPS cells].
Rinsho shinkeigaku = Clinical neurology - 1 Jan 2013
Egawa Naohiro, Inoue Haruhisa
Abstract excerpt
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder in which motor neuron (MN) loss in the spinal cord leads to progressive paralysis and death. Cytosolic aggregations in ALS MNs are composed of Tar DNA-binding protein-43 (TDP-43). Genetic analysis has identified more than twenty mutations of TDP-43 in ALS cases. Although accumulating evidence provides several hypotheses of disease mechanism, it...
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