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Modeling Parkinson’s Disease and Atypical Parkinsonian Syndromes Using Induced Pluripotent Stem Cells

Department of Neurology, Fukuoka University, Fukuoka 814-0180, Japan
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Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2018, 19(12), 3870; https://doi.org/10.3390/ijms19123870
Received: 3 September 2018 / Revised: 11 November 2018 / Accepted: 28 November 2018 / Published: 4 December 2018
(This article belongs to the Special Issue Disease Modeling Using Human Induced Pluripotent Stem Cells)
Parkinson’s disease (PD) and atypical parkinsonian syndromes are age-dependent multifactorial neurodegenerative diseases, which are clinically characterized by bradykinesia, tremor, muscle rigidity and postural instability. Although these diseases share several common clinical phenotypes, their pathophysiological aspects vary among the disease categories. Extensive animal-based approaches, as well as postmortem studies, have provided important insights into the disease mechanisms and potential therapeutic targets. However, the exact pathological mechanisms triggering such diseases still remain elusive. Furthermore, the effects of drugs observed in animal models are not always reproduced in human clinical trials. By using induced pluripotent stem cell (iPSC) technology, it has become possible to establish patient-specific iPSCs from their somatic cells and to effectively differentiate these iPSCs into different types of neurons, reproducing some key aspects of the disease phenotypes in vitro. In this review, we summarize recent findings from iPSC-based modeling of PD and several atypical parkinsonian syndromes including multiple system atrophy, frontotemporal dementia and parkinsonism linked to chromosome 17 and Perry syndrome. Furthermore, we discuss future challenges and prospects for modeling and understanding PD and atypical parkinsonian syndromes. View Full-Text
Keywords: induced pluripotent stem cells; Parkinson’s disease; atypical parkinsonian syndromes; multiple system atrophy; FTDP-17; Perry syndrome; proteinopathy; CRISPR induced pluripotent stem cells; Parkinson’s disease; atypical parkinsonian syndromes; multiple system atrophy; FTDP-17; Perry syndrome; proteinopathy; CRISPR
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Mishima, T.; Fujioka, S.; Fukae, J.; Yuasa-Kawada, J.; Tsuboi, Y. Modeling Parkinson’s Disease and Atypical Parkinsonian Syndromes Using Induced Pluripotent Stem Cells. Int. J. Mol. Sci. 2018, 19, 3870.

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