1. Brain aconitase activity is not decreased in progressive supranuclear palsy
- Author
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Lee-Cyn Ang, Yoshiaki Furukawa, Mark Guttman, Stephen J. Kish, Alex Rajput, C. L. Bamsey, and Paul S. Fitzmaurice
- Subjects
Aconitate Hydratase ,medicine.medical_specialty ,Central nervous system ,Brain ,Supranuclear ophthalmoplegia ,Human brain ,Biology ,Multiple System Atrophy ,medicine.disease ,Aconitase ,eye diseases ,Progressive supranuclear palsy ,Central nervous system disease ,Citric acid cycle ,medicine.anatomical_structure ,Degenerative disease ,Endocrinology ,Internal medicine ,medicine ,Humans ,Neurology (clinical) ,Supranuclear Palsy, Progressive ,Aged - Abstract
The novel finding of decreased activity of aconitase, a key Krebs cycle enzyme highly sensitive to oxidative damage, in cybrid cell lines using mitochondrial DNA from patients with progressive supranuclear palsy (PSP) implies an enzyme abnormality in brain. However, the authors found that postmortem brain aconitase activity is normal in PSP. This suggests that patients with PSP do not have systemic aconitase deficiency and that data derived from cybrid cell models of neurodegenerative disorders might not always predict similar changes in human brain.
- Published
- 2002