Molecular BiologybioRxivPreprint
Lipid mediated ER-stress contributes to the pathogenesis of mitochondrial myopathies
Inaya Laubach, Guido Primiano, Nneka Southwell…·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.