Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder affecting the motor nerves. At present, there is no effective therapy for this devastating disease and only one Food and Drug Administration (FDA)-approved drug, riluzole, is known to moderately extend survival. In the last decade, the field of ALS has made a remarkable leap forward in understanding some of the genetic causes of this disease and the role that different cell types play in the degenerative mechanism affecting motor neurons. In particular, astrocytes have been implicated in disease progression, and multiple studies suggest that these cells are valuable therapeutic targets. Recent technological advancements have provided new tools to generate astrocytes from ALS patients either from post-mortem biopsies or from skin fibroblasts through genetic reprogramming. The advent of induced pluripotent stem cell (iPSC) technology and the newly developed induced neural progenitor cells (iNPCs) have created unprecedented exciting opportunities to unravel the mechanisms involved in neurodegeneration and initiate high-throughput drug screenings.
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October 2014
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Conference Article|
September 18 2014
The non-cell-autonomous component of ALS: new in vitro models and future challenges
Laura Ferraiuolo
Laura Ferraiuolo
1
*The Research Institute at Nationwide Children's Hospital, Columbus, OH 43205, U.S.A.
1To whom correspondence should be addressed (email Laura.Ferraiuolo@nationwidechildrens.org).
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Publisher: Portland Press Ltd
Received:
June 10 2014
Online ISSN: 1470-8752
Print ISSN: 0300-5127
© The Authors Journal compilation © 2014 Biochemical Society
2014
Biochem Soc Trans (2014) 42 (5): 1270–1274.
Article history
Received:
June 10 2014
Citation
Laura Ferraiuolo; The non-cell-autonomous component of ALS: new in vitro models and future challenges. Biochem Soc Trans 1 October 2014; 42 (5): 1270–1274. doi: https://doi.org/10.1042/BST20140168
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