Article
Defective proteostasis in induced pluripotent stem cell models of frontotemporal lobar degeneration.
Translational psychiatry - 10 Dec 2022
Mahali Sidhartha, Martinez Rita, King Melvin, Verbeck Anthony, Harari Oscar, Benitez Bruno A, Horie Kanta, Sato Chihiro, Temple Sally, Karch Celeste M
Abstract excerpt
Impaired proteostasis is associated with normal aging and is accelerated in neurodegeneration. This impairment may lead to the accumulation of protein, which can be toxic to cells and tissue. In a subset of frontotemporal lobar degeneration with tau pathology (FTLD-tau) cases, pathogenic mutations in the microtubule-associated protein tau (MAPT) gene are sufficient to cause tau accumulation and neurodegeneration....
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