Article
Astrocytic reactivity triggered by defective autophagy and metabolic failure causes neurotoxicity in frontotemporal dementia type 3.
Stem cell reports - 9 Nov 2021
Chandrasekaran Abinaya, Dittlau Katarina Stoklund, Corsi Giulia I, Haukedal Henriette, Doncheva Nadezhda T, Ramakrishna Sarayu, Ambardar Sheetal, Salcedo Claudia, Schmidt Sissel I, Zhang Yu, Cirera Susanna, Pihl Maria, Schmid Benjamin, Nielsen Troels Tolstrup, Nielsen Jørgen E, Kolko Miriam, Kobolák Julianna, Dinnyés András, Hyttel Poul, Palakodeti Dasaradhi, Gorodkin Jan, Muddashetty Ravi S, Meyer Morten, Aldana Blanca I, Freude Kristine K
Abstract excerpt
Frontotemporal dementia type 3 (FTD3), caused by a point mutation in the charged multivesicular body protein 2B (CHMP2B), affects mitochondrial ultrastructure and the endolysosomal pathway in neurons. To dissect the astrocyte-specific impact of mutant CHMP2B expression, we generated astrocytes from human induced pluripotent stem cells (hiPSCs) and confirmed our findings in CHMP2B mutant mice. Our data provide...
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