Inhibition of RNA splicing triggers CHMP7 nuclear entry, impacting TDP-43 function and leading to the onset of ALS cellular phenotypes

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Al-Azzam et al. reveal that SmD1, a member of the SMN complex, regulates CHMP7 translocation. Reduced SmD1 expression in sporadic ALS leads to CHMP7 nuclear translocation, while overexpression prevents it, highlighting SmD1’s role in ALS pathogenesis.