Mitochondrial dysfunction in models of Progressive Supranuclear Palsy

Progressive Supranuclear Palsy (PSP) is a rare neurodegenerative disease hallmarked by tau pathology in both neurons and astrocytes. Familial forms of PSP are caused by mutations affecting tau (MAPT) while risk factors for sporadic PSP are largely unknown. Mitochondrial function declines during aging and mitochondrial dysfunction is observed in PSP. Generation of reactive oxygen species and mitochondrial dysfunction are thought to directly contribute to the production of tau deposits. Here we interrogated mitochondrial function in astrocytes and neurons derived from induced pluripotent stem cell models of sporadic and familial PSP.