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.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Amyotrophic Lateral Sclerosis / genetics
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Amyotrophic Lateral Sclerosis / pathology*
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Astrocytes / physiology
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Cell Culture Techniques*
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Cell Differentiation
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DNA-Binding Proteins / genetics
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Humans
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Induced Pluripotent Stem Cells
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Microglia / physiology
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RNA-Binding Protein FUS / genetics
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Superoxide Dismutase / genetics
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Superoxide Dismutase-1
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Vesicular Transport Proteins / genetics
Substances
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DNA-Binding Proteins
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FUS protein, human
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RNA-Binding Protein FUS
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SOD1 protein, human
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TARDBP protein, human
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VAPB protein, human
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Vesicular Transport Proteins
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Superoxide Dismutase
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Superoxide Dismutase-1