Altered energy metabolism in Fatal Familial Insomnia cerebral organoids is associated with astrogliosis and neuronal dysfunction

Fatal familial insomnia (FFI) is a rare neurodegenerative disease caused by a dominantly inherited single amino acid substitution (D178N) within the prion protein (PrP). No in vitro human brain tissue model for this disease has previously been available. Consequently, how this mutation exerts its da...

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Detalles Bibliográficos
Autores principales: Foliaki, Simote T., Smith, Anna, Schwarz, Benjamin, Bohrnsen, Eric, Bosio, Catharine M., Williams, Katie, Orrú, Christina D., Lachenauer, Hailey, Groveman, Bradley R., Haigh, Cathryn L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2023
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9851538/
https://www.ncbi.nlm.nih.gov/pubmed/36656833
http://dx.doi.org/10.1371/journal.pgen.1010565

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9851538/
https://www.ncbi.nlm.nih.gov/pubmed/36656833
http://dx.doi.org/10.1371/journal.pgen.1010565

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