Modelling Parkinson’s Disease: iPSCs towards Better Understanding of Human Pathology

Parkinson’s Disease (PD) is a chronic neurodegenerative disorder characterized by motor and non-motor symptoms, among which are bradykinesia, rigidity, tremor as well as mental symptoms such as dementia. The underlying cause of Parkinson disease is degeneration of dopaminergic neurons. It has been c...

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Main Authors: Sahar Avazzadeh, Jara Maria Baena, Cameron Keighron, Yajaira Feller-Sanchez, Leo R. Quinlan
Format: Article
Language:English
Published: MDPI AG 2021-03-01
Series:Brain Sciences
Subjects:
Online Access:https://www.mdpi.com/2076-3425/11/3/373
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author Sahar Avazzadeh
Jara Maria Baena
Cameron Keighron
Yajaira Feller-Sanchez
Leo R. Quinlan
author_facet Sahar Avazzadeh
Jara Maria Baena
Cameron Keighron
Yajaira Feller-Sanchez
Leo R. Quinlan
author_sort Sahar Avazzadeh
collection DOAJ
description Parkinson’s Disease (PD) is a chronic neurodegenerative disorder characterized by motor and non-motor symptoms, among which are bradykinesia, rigidity, tremor as well as mental symptoms such as dementia. The underlying cause of Parkinson disease is degeneration of dopaminergic neurons. It has been challenging to develop an efficient animal model to accurately represent the complex phenotypes found with PD. However, it has become possible to recapitulate the myriad of phenotypes underlying the PD pathology by using human induced pluripotent stem cell (iPSC) technology. Patient-specific iPSC-derived dopaminergic neurons are available and present an opportunity to study many aspects of the PD phenotypes in a dish. In this review, we report the available data on iPSC-derived neurons derived from PD patients with identified gene mutations. Specifically, we will report on the key phenotypes of the generated iPSC-derived neurons from PD patients with different genetic background. Furthermore, we discuss the relationship these cellular phenotypes have to PD pathology and future challenges and prospects for iPSC modelling and understanding of the pathogenesis of PD.
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spelling doaj.art-605802c2b72844fa9e575786bfa0db962023-11-21T10:29:08ZengMDPI AGBrain Sciences2076-34252021-03-0111337310.3390/brainsci11030373Modelling Parkinson’s Disease: iPSCs towards Better Understanding of Human PathologySahar Avazzadeh0Jara Maria Baena1Cameron Keighron2Yajaira Feller-Sanchez3Leo R. Quinlan4Physiology and CÚRAM SFI Centre for Research in Medical Devices, School of Medicine, National University of Ireland (NUI), H91 TK33 Galway, IrelandPhysiology and CÚRAM SFI Centre for Research in Medical Devices, School of Medicine, National University of Ireland (NUI), H91 TK33 Galway, IrelandPhysiology and CÚRAM SFI Centre for Research in Medical Devices, School of Medicine, National University of Ireland (NUI), H91 TK33 Galway, IrelandPhysiology and CÚRAM SFI Centre for Research in Medical Devices, School of Medicine, National University of Ireland (NUI), H91 TK33 Galway, IrelandPhysiology and CÚRAM SFI Centre for Research in Medical Devices, School of Medicine, National University of Ireland (NUI), H91 TK33 Galway, IrelandParkinson’s Disease (PD) is a chronic neurodegenerative disorder characterized by motor and non-motor symptoms, among which are bradykinesia, rigidity, tremor as well as mental symptoms such as dementia. The underlying cause of Parkinson disease is degeneration of dopaminergic neurons. It has been challenging to develop an efficient animal model to accurately represent the complex phenotypes found with PD. However, it has become possible to recapitulate the myriad of phenotypes underlying the PD pathology by using human induced pluripotent stem cell (iPSC) technology. Patient-specific iPSC-derived dopaminergic neurons are available and present an opportunity to study many aspects of the PD phenotypes in a dish. In this review, we report the available data on iPSC-derived neurons derived from PD patients with identified gene mutations. Specifically, we will report on the key phenotypes of the generated iPSC-derived neurons from PD patients with different genetic background. Furthermore, we discuss the relationship these cellular phenotypes have to PD pathology and future challenges and prospects for iPSC modelling and understanding of the pathogenesis of PD.https://www.mdpi.com/2076-3425/11/3/373Parkinson’s diseaseinduced pluripotent stem cellshuman pathology
spellingShingle Sahar Avazzadeh
Jara Maria Baena
Cameron Keighron
Yajaira Feller-Sanchez
Leo R. Quinlan
Modelling Parkinson’s Disease: iPSCs towards Better Understanding of Human Pathology
Brain Sciences
Parkinson’s disease
induced pluripotent stem cells
human pathology
title Modelling Parkinson’s Disease: iPSCs towards Better Understanding of Human Pathology
title_full Modelling Parkinson’s Disease: iPSCs towards Better Understanding of Human Pathology
title_fullStr Modelling Parkinson’s Disease: iPSCs towards Better Understanding of Human Pathology
title_full_unstemmed Modelling Parkinson’s Disease: iPSCs towards Better Understanding of Human Pathology
title_short Modelling Parkinson’s Disease: iPSCs towards Better Understanding of Human Pathology
title_sort modelling parkinson s disease ipscs towards better understanding of human pathology
topic Parkinson’s disease
induced pluripotent stem cells
human pathology
url https://www.mdpi.com/2076-3425/11/3/373
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