Journal
BRAIN RESEARCH
Volume 1656, Issue -, Pages 88-97Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.brainres.2015.10.003
Keywords
Induced pluripotent stem cells (iPSCs); Amyotrophic lateral sclerosis (ALS); Frontotemporal dementia (FM); Frontotemporal lobar degeneration (FTLD)
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Funding
- National Institute of Health [0D011915]
- Veterans Administrations BLR&D Merit Review Award [I01 BX0011-8]
- Pilot Award [I21 BX1625]
- Muscular Dystrophy Association [217592]
- Consortium for Frontotemporal Dementia Research (CFR)
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Recent advances in genetics and neuropathology support the idea that amyotrophic lateral sclerosis (ALS) and frontotemporal lobar dementia (FTD) are two ends of a disease spectrum. Although several animal models have been developed to investigate the pathogenesis and disease progression in ALS and FTD, there are significant limitations that hamper our ability to connect these models with the neurodegenerative processes in human diseases. With the technical breakthrough in reprogramming biology, it is now possible to generate patient-specific induced pluripotent stem cells (iPSCs) and disease-relevant neuron subtypes. This review provides a comprehensive summary of studies that use iPSC-derived neurons to model ALS and FTD. We discuss the unique capabilities of iPSC-derived neurons that capture some key features of ALS and FTD, and underscore their potential roles in drug discovery. There are, however, several critical caveats that require improvements before iPSC-derived neurons can become highly effective disease models. Published by Elsevier B.V.
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