Review: Induced pluripotent stem cell models of frontotemporal dementia.
The increasing prevalence of dementia in the ageing population combined with the lack of treatments and the burden on national health care systems globally make dementia a public health priority. Despite the plethora of important research findings published over the past two decades, the mechanisms underlying dementia are still poorly understood and the progress in pharmacological interventions is limited. Recent advances in cellular reprogramming and genome engineering technologies offer an unprecedented new paradigm in disease modeling. Induced pluripotent stem cells (iPSCs) have enabled the study of patient-derived neurons in vitro, a significant progress in the field of dementia research. The first studies using iPSCs to model dementia have recently emerged, holding promise for elucidating disease pathogenic mechanisms and accelerating drug discovery. In this review, we summarize the major findings of iPSC-based studies in frontotemporal dementia (FTD) and FTD overlapping with amyotrophic lateral sclerosis (FTD/ALS). We also discuss some of the main challenges in the use of iPSCs to model complex, late-onset neurodegenerative diseases such as dementias.
Department of Molecular Neuroscience, UCL Institute of Neurology, London, WC1N 1PJ, UK. e.preza@ucl.ac.uk.
E
E
Preza
Department of Molecular Neuroscience, UCL Institute of Neurology, London, WC1N 1PJ, UK.
J
J
Hardy
Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, London, WC1N 1PJ, UK.
T
T
Warner
Department of Molecular Neuroscience, UCL Institute of Neurology, London, WC1N 1PJ, UK.
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S
Wray
D057180:Frontotemporal Dementia; D006801:Humans; D066298:In Vitro Techniques; D057026:Induced Pluripotent Stem Cells; D008954:Models, Biological
FTD/ALS; amyotrophic lateral sclerosis; dementia modeling; frontotemporal dementia; induced pluripotent stem cells