Inhibiting Cellular Stress Response May Normalize Processes, Function In Patients With Rare ALS Type, Study Finds

ALS News Today (8/7, Levinson) reports a study found that “inhibiting a cellular stress response can normalize processes and function” in motor neurons for patients with “ALS related to the P56S mutation in the VAPB gene, a rare genetic abnormality.” According to the article, disrupting the mitochondrial-associated membrane (ER MAM) “can contribute to cellular stress and activate a cell’s integrated stress response (ISR). This is what the team saw in the motor neurons – sensitivity to stress was higher and adaptation to it was impaired in mutated cells relative to healthy ones. The ISR can initially be helpful by enabling a cell to adapt to a diverse stimuli and changes in gene activity. Over time, however, it may lead to cell damage.” Although the findings are specific to VAPB-associated ALS, they “may have broader applicability in ALS and suggest the importance of considering genetic makeup in clinical trials.” The study was published in EMBO Molecular Medicine.