Cargando…

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...

Descripción completa

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

Ejemplares similares

Sporadic Creutzfeldt-Jakob disease prion infection of human cerebral organoids
por: Groveman, Bradley R., et al.
Publicado: (2019)

A PrP EGFR signaling axis controls neural stem cell senescence through modulating cellular energy pathways
por: Groveman, Bradley R., et al.
Publicado: (2023)

Neural cell engraftment therapy for sporadic Creutzfeldt-Jakob disease restores neuroelectrophysiological parameters in a cerebral organoid model
por: Williams, Katie, et al.
Publicado: (2023)

Neuronal excitatory-to-inhibitory balance is altered in cerebral organoid models of genetic neurological diseases
por: Foliaki, Simote T., et al.
Publicado: (2021)

Sporadic Creutzfeldt–Jakob disease infected human cerebral organoids retain the original human brain subtype features following transmission to humanized transgenic mice
por: Groveman, Bradley R., et al.
Publicado: (2023)

Stress and viral insults do not trigger E200K PrP conversion in human cerebral organoids
por: Smith, Anna, et al.
Publicado: (2022)

Pathogenic Prion Protein Isoforms Are Not Present in Cerebral Organoids Generated from Asymptomatic Donors Carrying the E200K Mutation Associated with Familial Prion Disease
por: Foliaki, Simote T., et al.
Publicado: (2020)

Human cerebral organoids as a therapeutic drug screening model for Creutzfeldt–Jakob disease
por: Groveman, Bradley R., et al.
Publicado: (2021)

Reduced SOD2 expression does not influence prion disease course or pathology in mice
por: Foliaki, Simote T., et al.
Publicado: (2021)

Cerebral organoids as a new model for prion disease
por: Groveman, Bradley R., et al.
Publicado: (2021)

Prion propagation and cellular dysfunction in prion disease: Disconnecting the dots
por: Foliaki, Simote T., et al.
Publicado: (2023)

Temporary alteration of neuronal network communication is a protective response to redox imbalance that requires GPI-anchored prion protein
por: Foliaki, Simote T., et al.
Publicado: (2023)

Using our mini-brains: cerebral organoids as an improved cellular model for human prion disease
por: Groveman, Bradley R., et al.
Publicado: (2019)

Hereditary E200K mutation within the prion protein gene alters human iPSC derived cardiomyocyte function
por: Wood, Aleksandar R., et al.
Publicado: (2022)

Route of Francisella tularensis infection informs spatiotemporal metabolic reprogramming and inflammation in mice
por: Jessop, Forrest, et al.
Publicado: (2023)

Targeting 2-Oxoglutarate–Dependent Dioxygenases Promotes Metabolic Reprogramming That Protects against Lethal SARS-CoV-2 Infection in the K18-hACE2 Transgenic Mouse Model
por: Jessop, Forrest, et al.
Publicado: (2023)

Prion protein cleavage fragments regulate adult neural stem cell quiescence through redox modulation of mitochondrial fission and SOD2 expression
por: Collins, Steven J., et al.
Publicado: (2018)

Impairing RAGE signaling promotes survival and limits disease pathogenesis following SARS-CoV-2 infection in mice
por: Jessop, Forrest, et al.
Publicado: (2022)

Cortical superficial siderosis is associated with reactive astrogliosis in cerebral amyloid angiopathy
por: Auger, Corinne A., et al.
Publicado: (2023)

Astrogenesis versus astrogliosis
por: Koob, Andrew O.
Publicado: (2017)

Neuritin inhibits astrogliosis to ameliorate diabetic cognitive dysfunction
por: Zhang, Zuo, et al.
Publicado: (2021)

Reactive astrogliosis is associated with higher cerebral glucose consumption in the early Alzheimer’s continuum
por: Salvadó, Gemma, et al.
Publicado: (2022)

Region-specific astrogliosis: differential vessel formation contributes to different patterns of astrogliosis in the cortex and striatum
por: Yang, Haijie, et al.
Publicado: (2020)

Defining the Prion Type of Fatal Familial Insomnia
por: Jürgens-Wemheuer, Wiebke, et al.
Publicado: (2021)

Beneficial effect of agmatine on brain apoptosis, astrogliosis, and edema after rat transient cerebral ischemia
por: Wang, Che-Chuan, et al.
Publicado: (2010)

Crystallin Alpha-B Overexpression as a Possible Marker of Reactive Astrogliosis in Human Cerebral Contusions
por: Becerra-Hernández, Lina Vanessa, et al.
Publicado: (2022)

Proposal of new diagnostic criteria for fatal familial insomnia
por: Chu, Min, et al.
Publicado: (2022)

Scutellarin regulates microglia-mediated TNC1 astrocytic reaction and astrogliosis in cerebral ischemia in the adult rats
por: Fang, Ming, et al.
Publicado: (2015)

Sulfasalazine decreases astrogliosis‐mediated seizure burden
por: Alcoreza, Oscar, et al.
Publicado: (2022)

Imaging of Reactive Astrogliosis by Positron Emission Tomography
por: Harada, Ryuichi, et al.
Publicado: (2022)

Reactive astrogliosis in the era of single-cell transcriptomics
por: Matusova, Zuzana, et al.
Publicado: (2023)

Cerebral Asymmetry in Insomnia Sufferers
por: St-Jean, Geneviève, et al.
Publicado: (2012)

A proposal of new diagnostic pathway for fatal familial insomnia
por: Krasnianski, A, et al.
Publicado: (2014)

Expert Consensus on Clinical Diagnostic Criteria for Fatal Familial Insomnia
por: Wu, Li-Yong, et al.
Publicado: (2018)

Dysfunction of the cardiac parasympathetic system in fatal familial insomnia: a heart rate variability study
por: Cui, Yue, et al.
Publicado: (2022)

Non-invasive quantification of brain tumor-induced astrogliosis
por: Lee, Jisook, et al.
Publicado: (2011)

The Role of Astrogliosis in Formation of the Syrinx in Spinal Cord Injury
por: Kwiecien, Jacek M., et al.
Publicado: (2021)

Diversity of Reactive Astrogliosis in CNS Pathology: Heterogeneity or Plasticity?
por: Moulson, Aaron J., et al.
Publicado: (2021)

Corrigendum: Reactive astrogliosis in the era of single-cell transcriptomics
por: Matusova, Zuzana, et al.
Publicado: (2023)

The moral status of cerebral organoids
por: Hostiuc, Sorin, et al.
Publicado: (2019)

Cannot write session to /tmp/vufind_sessions/sess_k0sn9co9q4tmaqptvrh6s3jn8m