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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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Autores principales: Avazzadeh, Sahar, Baena, Jara Maria, Keighron, Cameron, Feller-Sanchez, Yajaira, Quinlan, Leo R.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8000082/
https://www.ncbi.nlm.nih.gov/pubmed/33799491
http://dx.doi.org/10.3390/brainsci11030373
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author Avazzadeh, Sahar
Baena, Jara Maria
Keighron, Cameron
Feller-Sanchez, Yajaira
Quinlan, Leo R.
author_facet Avazzadeh, Sahar
Baena, Jara Maria
Keighron, Cameron
Feller-Sanchez, Yajaira
Quinlan, Leo R.
author_sort Avazzadeh, Sahar
collection PubMed
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 pubmed-80000822021-03-28 Modelling Parkinson’s Disease: iPSCs towards Better Understanding of Human Pathology Avazzadeh, Sahar Baena, Jara Maria Keighron, Cameron Feller-Sanchez, Yajaira Quinlan, Leo R. Brain Sci Review 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. MDPI 2021-03-14 /pmc/articles/PMC8000082/ /pubmed/33799491 http://dx.doi.org/10.3390/brainsci11030373 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) ).
spellingShingle Review
Avazzadeh, Sahar
Baena, Jara Maria
Keighron, Cameron
Feller-Sanchez, Yajaira
Quinlan, Leo R.
Modelling Parkinson’s Disease: iPSCs towards Better Understanding of 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 Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8000082/
https://www.ncbi.nlm.nih.gov/pubmed/33799491
http://dx.doi.org/10.3390/brainsci11030373
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