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Pluripotent stem cells for uncovering the role of mitochondria in human brain function and dysfunction

Item Type:Review
Title:Pluripotent stem cells for uncovering the role of mitochondria in human brain function and dysfunction
Creators Name:Zink, A. and Priller, J. and Prigione, A.
Abstract:Mitochondrial dysfunctions are a known pathogenetic mechanism of a number of neurological and psychiatric disorders. At the same time, mutations in genes encoding for components of the mitochondrial respiratory chain cause mitochondrial diseases, which commonly exhibit neurological symptoms. Mitochondria are therefore critical for the functionality of the human nervous system. The importance of mitochondria stems from their key roles in cellular metabolism, calcium handling, redox and protein homeostasis, and overall cellular homeostasis through their dynamic network. Here, we describe how the use of pluripotent stem cells (PSCs) may help addressing the physiological and pathological relevance of mitochondria for the human nervous system. PSCs allow the generation of patient-derived neurons and glia and the identification of gene-specific and mutation-specific cellular phenotypes via genome engineering approaches. We discuss the recent advances in PSC-based modeling of brain diseases and the current challenges of the field. We anticipate that the careful use of PSCs will improve our understanding of the impact of mitochondria in neurological and psychiatric disorders and the search for effective therapeutic avenues.
Keywords:Mitochondria, iPSCs, Genome Editing, Neurological Disorders, Mitochondrial Diseases
Source:Journal of Molecular Biology
ISSN:0022-2836
Publisher:Elsevier (The Netherlands)
Volume:430
Number:7
Page Range:891-903
Date:30 March 2018
Official Publication:https://doi.org/10.1016/j.jmb.2018.02.005
PubMed:View item in PubMed

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