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Review
. 2015;14(23):3679-88.
doi: 10.1080/15384101.2015.1093712.

A perspective on stem cell modeling of amyotrophic lateral sclerosis

A Sophie de Boer  1   2 , Kevin Eggan  1
Affiliations
Review

A perspective on stem cell modeling of amyotrophic lateral sclerosis

A Sophie de Boer et al. Cell Cycle. 2015.

Abstract

Amyotrophic lateral sclerosis is a complex neurodegenerative disease. Limitations in animal models have impeded progress in studying disease pathology and potential drug discovery. Here, we will review recent advances in the development of stem cell models for the study of ALS. Additionally, we will discuss the progress toward therapeutic development derived from these stem cell based assays.

Keywords: Amyotrophic Lateral Sclerosis; astrocyte; embryonic stem cells; glia; induced pluripotent stem cells; microglia; motor neuron.

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Figures

Figure 1.
Figure 1.
Schematic overview of cell autonomous phenotypes found stem cell models of ALS (A) Schematic overview of iPSC derivation. (B-E) Schematic overview of phenotypes found in SOD1, TDP-43, C9ORF72 and Sporadic iPSC derived motor neurons.
Figure 2.
Figure 2.
Schematic overview of non-cell autonomous phenotypes found in stem cell models of ALS. (A-D) Schematic overview of phenotypes found in SOD1, TDP-43, C9ORF72 and Sporadic astrocytes.
See this image and copyright information in PMC

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