Lipid mediated ER-stress contributes to the pathogenesis of mitochondrial myopathies
Inaya Laubach, Guido Primiano, Nneka Southwell, Nicola Rizzardi, Belem Yoval‐Sánchez, Christian Bergamini, Serenella Servidei, Alexander S. Galkin, Giovanni Manfredi, Qiuying Chen, Marilena D’Aurelio
This study identifies lipid-mediated ER stress as a driver of muscle dysfunction in mitochondrial myopathies and shows that ceramide inhibition improves symptoms in a mouse model.
bioRxiv · Jun 12, 2026 · PreprintMolecular Biology