Article
Hypoxic stress is an early pathogenic event in human VCP-mutant ALS astrocytes.
Stem cell reports - 13 Jan 2026
Franklin Hannah D, Crerar Hamish, Parnandi Nishita, Lattke Michael, Majewski Stanislaw, Clarke Benjamin E, Pallikonda Husayn, Howell Michael, Boulton Simon J, Patani Rickie
Abstract excerpt
Astrocytes are essential regulators of neuronal health, and their dysfunction contributes to neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS). Using human induced pluripotent stem cell (iPSC)-derived astrocytes carrying ALS-associated VCP mutations, we uncover cell-autonomous activation of the hypoxia response under basal conditions. VCP-mutant astrocytes exhibit increased nuclear...
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