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Necroptosis Drives Motor Neuron Death in Models of Both Sporadic and Familial ALS

6 Feb 2014

Abstract excerpt

Most cases of neurodegenerative diseases are sporadic, hindering the use of genetic mouse models to analyze disease mechanisms. Focusing on the motor neuron (MN) disease amyotrophic lateral sclerosis (ALS), we therefore devised a fully humanized coculture model composed of human adult primary sporadic ALS (sALS) astrocytes and human embryonic stem-cell-derived MNs. The model reproduces the cardinal features of...

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PMID: 24508385
DOI: 10.1016/j.neuron.2014.01.011

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