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

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...

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Detalles Bibliográficos
Autores principales: Inak, Gizem, Rybak-Wolf, Agnieszka, Lisowski, Pawel, Pentimalli, Tancredi M., 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, Mayr, Johannes A., Wortmann, Saskia B., Priller, Josef, Gotthardt, Michael, Jones, Dean P., Mayatepek, Ertan, Stenzel, Werner, Diecke, Sebastian, Kühn, Ralf, Wanker, Erich E., Rajewsky, Nikolaus, Schuelke, Markus, Prigione, Alessandro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2021
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7997884/
https://www.ncbi.nlm.nih.gov/pubmed/33771987
http://dx.doi.org/10.1038/s41467-021-22117-z

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