Generation of human brain organoids for mitochondrial disease modeling

Item Type:	Article
Title:	Generation of human brain organoids for mitochondrial disease modeling
Creators Name:	Le, S. and Petersilie, L. and Inak, G. and Menacho-Pando, C. and Kafitz, K.W. and Rybak-Wolf, A. and Rajewsky, N. and Rose, C.R. and Prigione, A.
Abstract:	Mitochondrial diseases represent the largest class of inborn errors of metabolism and are currently incurable. These diseases cause neurodevelopmental defects whose underlying mechanisms remain to be elucidated. A major roadblock is the lack of effective models recapitulating the early-onset neuronal impairment seen in the patients. Advances in the technology of induced pluripotent stem cells (iPSCs) enable the generation of three-dimensional (3D) brain organoids that can be used to investigate the impact of diseases on the development and organization of the nervous system. Researchers, including these authors, have recently introduced human brain organoids to model mitochondrial disorders. This paper reports a detailed protocol for the robust generation of human iPSC-derived brain organoids and their use in mitochondrial bioenergetic profiling and imaging analyses. These experiments will allow the use of brain organoids to investigate metabolic and developmental dysfunctions and may provide crucial information to dissect the neuronal pathology of mitochondrial diseases.
Keywords:	Brain, Cell Differentiation, Induced Pluripotent Stem Cells, Mitochondrial Diseases, Organoids
Source:	Journal of Visualized Experiments
ISSN:	1940-087X
Publisher:	JoVE
Number:	172
Page Range:	e62756
Date:	21 June 2021
Official Publication:	https://doi.org/10.3791/62756
PubMed:	View item in PubMed

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