Brief Communication
Further Evidence for Mitochondrial Dysfunction in Progressive Supranuclear Palsy

https://doi.org/10.1006/exnr.2000.7607Get rights and content

Abstract

Recent data from our laboratory have identified a role for mitochondrial dysfunction in the pathogenesis of progressive supranuclear palsy (PSP). To extend this finding, we measured key parameters of mitochondrial function in platelet-derived cytoplasmic hybrid (cybrid) cell lines expressing mitochondrial genes from patients with PSP. We observed significant decreases in aconitase activity, cellular ATP levels, and oxygen consumption in PSP cybrids as compared to control cybrids, further suggesting a contributory role of impaired mitochondrial energy metabolism in PSP, possibly due to genetic abnormalities of mitochondrial DNA.

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To whom correspondence should be addressed at Department of Neurology and Neuroscience, Room A-503, Weill Medical College of Cornell University, 525 East 68th Street, New York, NY 10021. Fax: (212) 746-8276. E-mail: [email protected].

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