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Convergent activation of the integrated stress response and ER–mitochondria uncoupling in VAPB-associated ALS

5 Aug 2025

Abstract excerpt

Vesicle-associated membrane protein-associated protein-B (VAPB) is an endoplasmic reticulum (ER) membrane-bound protein. The P56S mutation in VAPB causes a dominant, familial form of amyotrophic lateral sclerosis (ALS). However, the mechanism by which this mutation leads to motor neuron (MN) degeneration remains unclear. Utilizing inducible pluripotent stem cell (iPSC)-derived MNs expressing either wild-type (WT)...

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PMID: 40764463
DOI: 10.1038/s44321-025-00279-3

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