Further evidence for mitochondrial dysfunction in progressive supranuclearpalsy

Recent data from our laboratory have identified a role for mitochondrial dy sfunction in the pathogenesis of progressive supranuclear palsy (PSP). To e xtend this finding, we measured key parameters of mitochondrial function in platelet-derived cytoplasmic hybrid (cybrid) cell lines expressing mitocho ndrial genes from patients with PSP, We observed significant decreases in a conitase activity, cellular ATP levels, and oxygen consumption in PSP cybri ds as compared to control cybrids, further suggesting a contributory role o f impaired mitochondrial energy metabolism in PSP, possibly due to genetic abnormalities of mitochondrial DNA. (C) 2001 Academic Press.