Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome

Inak, Gizem; Rybak‐Wolf, Agnieszka; Lisowski, Paweł; Massimo Pentimalli, Tancredi; Jüttner, René; Glažar, Petar; Uppal, Karan; Bottani, Emanuela; Brunetti, Dario; Secker, Christopher; Zink, Annika; Meierhofer, David; Henke, Marie‐Thérèse; Dey, Monishita; Ciptasari, Ummi; Mlody, Barbara; Hahn, Tobias; Berruezo-Llacuna, Maria; Karaiskos, Nikos; Di Virgilio, Michela; A. Mayr, Johannes; B. Wortmann, Saskia; Priller, Josef; Gotthardt, Michael; P. Jones, Dean; Mayatepek, Ertan; Stenzel, Werner; Diecke, Sebastian; Kühn, Ralf; E. Wanker, Erich; Rajewsky, Nikolaus; Schuelke, Markus; Prigione, Alessandro

doi:10.1038/s41467-021-22117-z

Public

Defective metabolic programming impairs early neuronal morphogenesis in neural cultures and an organoid model of Leigh syndrome

Published in Nature Communications • Mar 26, 2021

Authors:

Gizem Inak

Agnieszka Rybak‐Wolf

Paweł Lisowski

Abstract

Leigh syndrome (LS) is a severe manifestation of mitochondrial disease in children and is currently incurable. The lack of effective models hampers our understanding of the mechanisms underlying the neuronal pathology of LS. Using patient-derived induced pluripotent stem cells and CRISPR/Cas9 engine...

Subject

Morphogenesis

Biology

Organoid

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