Article

Mitochondrial dysfunction heightens the integrated stress response to drive ALS pathogenesis

2024-05-14

Abstract excerpt

<h4>ABSTRACT</h4> Vesicle-associated membrane protein-associated protein-B (VAPB) is an ER membrane bound protein. VAPB P56S causes a dominant, familial form of amyotrophic lateral sclerosis (ALS), however, the mechanism through which this mutation causes motor neuron (MN) disease remains unknown. Using inducible wild type (WT) and VAPB P56S expressing iPSC-derived MNs we show that VAPB P56S, but not WT, protein...

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Literature Corpus work: 773d6c49-3fcc-5c42-847a-41a3f99b2d05
DOI: 10.1101/2024.05.13.594000

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