title: Further evidence for mitochondrial dysfunction in progressive supranuclear palsy.
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.
authors.affiliation: Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York, New York 10021, USA. daa2010@mail.med.cornell.edu
authors.forename: D S
authors.initials: DS
authors.lastname: Albers
authors.forename: R H
authors.initials: RH
authors.lastname: Swerdlow
authors.forename: G
authors.initials: G
authors.lastname: Manfredi
authors.forename: C
authors.initials: C
authors.lastname: Gajewski
authors.forename: L
authors.initials: L
authors.lastname: Yang
authors.forename: W D
authors.initials: WD
authors.lastname: Parker
authors.forename: M F
authors.initials: MF
authors.lastname: Beal
mesh_terms: D000255:Adenosine Triphosphate / Q000378:metabolism; D000368:Aged; D001792:Blood Platelets / Q000473:pathology / Q000502:physiology*; D002459:Cell Fusion; D005260:Female; D006801:Humans; D006822:Hybrid Cells; D008297:Male; D008928:Mitochondria / Q000378:metabolism*; D009447:Neuroblastoma; D010101:Oxygen Consumption; D012016:Reference Values; D013494:Supranuclear Palsy, Progressive / Q000097:blood / Q000378:metabolism*; D014407:Tumor Cells, Cultured
chemical_list: D000255:Adenosine Triphosphate