Further evidence for mitochondrial dysfunction in progressive supranuclear palsy

被引：62

作者：

Albers, DS

Swerdlow, RH

Manfredi, G

Gajewski, C

Yang, LC

Parker, WD

Beal, MF

机构：

[1] Cornell Univ, Weill Med Coll, Dept Neurol & Neurosci, New York, NY 10021 USA

[2] Univ Virginia, Dept Neurol, Charlottesville, VA USA

[3] Univ Virginia, Ctr Study Neurogenerat Dis, Charlottesville, VA USA

来源：

EXPERIMENTAL NEUROLOGY | 2001年 / 168卷 / 01期

关键词：

neurodegeneration; cybrids; ATP; oxygen; aconitase;

D O I：

10.1006/exnr.2000.7607

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

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. (C) 2001 Academic Press.

引用

页码：196 / 198

页数：3

共 22 条

[1] Frontal lobe dysfunction in progressive supranuclear palsy: Evidence for oxidative stress and mitochondrial impairment [J].