Review: Induced pluripotent stem cell models of frontotemporal dementia. (October 2016)

• Record Type: Journal Article
• Authors: Preza, E.; Hardy, J.; Warner, T.; Wray, S.
• Is Part Of: Neuropathology & applied neurobiology. Volume 42:Number 6(2016)
• Page Start: 497
• Page End: 520
• Publication Date: 2016-10
• Subjects: amyotrophic lateral sclerosis -- dementia modeling -- frontotemporal dementia -- FTD/ALS -- induced pluripotent stem cells; Nervous system -- Diseases -- Pathology -- Periodicals; Nervous system -- Diseases -- Periodicals; 616.8
• DOI: 10.1111/nan.12334
• Languages: English
• ISSNs: 0305-1846

Abstract

Abstract : 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. Abstract : Preza et al . provide an overview of the emerging field of disease modelling using induced pluripotent stem cells (iPSCs) allowing the study of patient‐derived neurons in vitro. The paper considers the major contributions in the field of FTD and FTD with amyotrophic lateral sclerosis.