Expression of mutant exon 1 huntingtin fragments in human neural stem cells and neurons causes inclusion formation and mitochondrial dysfunction

Robust cellular models are key in determining pathological mechanisms that lead to neurotoxicity in Huntington's disease (HD) and for high throughput pre‐clinical screening of potential therapeutic compounds. Such models exist but mostly comprise non‐human or non‐neuronal cells that may not rec...

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Detalles Bibliográficos
Autores principales: Ghosh, Rhia, Wood‐Kaczmar, Alison, Dobson, Lucianne, Smith, Edward J., Sirinathsinghji, Eva C., Kriston‐Vizi, Janos, Hargreaves, Iain P., Heaton, Robert, Herrmann, Frank, Abramov, Andrey Y., Lam, Amanda J., Heales, Simon J., Ketteler, Robin, Bates, Gillian P., Andre, Ralph, Tabrizi, Sarah J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2020
Materias:
Research Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8432155/
https://www.ncbi.nlm.nih.gov/pubmed/32329133
http://dx.doi.org/10.1096/fj.201902277RR

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8432155/
https://www.ncbi.nlm.nih.gov/pubmed/32329133
http://dx.doi.org/10.1096/fj.201902277RR

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