Abstract:
:It has been proposed that human embryonic stem cells could be used to provide an inexhaustible supply of differentiated cell types for the study of disease processes. Although methods for differentiating embryonic stem cells into specific cell types have become increasingly sophisticated, the utility of the resulting cells for modeling disease has not been determined. We have asked whether specific neuronal subtypes produced from human embryonic stem cells can be used to investigate the mechanisms leading to neural degeneration in amyotrophic lateral sclerosis (ALS). We show that human spinal motor neurons, but not interneurons, are selectively sensitive to the toxic effect of glial cells carrying an ALS-causing mutation in the SOD1 gene. Our findings demonstrate the relevance of these non-cell-autonomous effects to human motor neurons and more broadly demonstrate the utility of human embryonic stem cells for studying disease and identifying potential therapeutics.
journal_name
Cell Stem Celljournal_title
Cell stem cellauthors
Di Giorgio FP,Boulting GL,Bobrowicz S,Eggan KCdoi
10.1016/j.stem.2008.09.017subject
Has Abstractpub_date
2008-12-04 00:00:00pages
637-48issue
6eissn
1934-5909issn
1875-9777pii
S1934-5909(08)00522-5journal_volume
3pub_type
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