Ataxia Telangiectasia patient-derived neuronal and brain organoid models reveal mitochondrial dysfunction and oxidative stress

Ataxia Telangiectasia (AT) is a rare disorder caused by mutations in the ATM gene and results in progressive neurodegeneration for reasons that remain poorly understood. In addition to its central role in nuclear DNA repair, ATM operates outside the nucleus to regulate metabolism, redox homeostasis...

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Autores principales:	Hannah C. Leeson, Julio Aguado, Cecilia Gómez-Inclán, Harman Kaur Chaggar, Atefah Taherian Fard, Zoe Hunter, Martin F. Lavin, Alan Mackay-Sim, Ernst J. Wolvetang
Formato:	Artigo
Lenguaje:	Inglês
Publicado:	Elsevier 2024-09-01
Colección:	Neurobiology of Disease
Materias:	Ataxia telangiectasia Brain organoids Mitochondria Neurodegeneration Cellular senescence Oxidative stress
Acceso en línea:	http://www.sciencedirect.com/science/article/pii/S096999612400161X
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