Abstract
The nature of mitochondrial dysfunction in dopaminergic neurons in familial Parkinson's disease (PD) is unknown. We characterized the pathophenotypes of dopaminergic neuronal cells that were deficient in PINK1 or DJ-1, genes with mutations linked to familial PD. Both PINK1- and DJ-1-deficient dopaminergic neurons had the increased production of ROS, severe mitochondrial structural damages and complex I deficits. A striking decrease in complex IV activity was also prominent by the PINK1-deficiency. The complex I deficits were relatively PD-specific and were significantly improved by an antioxidant Trolox. These data suggest that mitochondrial deficits are severe in dopaminergic neurons in familial PD and antioxidant-mediated functional recovery is feasible.
Original language | English |
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Pages (from-to) | 707-715 |
Number of pages | 9 |
Journal | Mitochondrion |
Volume | 11 |
Issue number | 5 |
DOIs | |
State | Published - Sep 2011 |
Bibliographical note
Funding Information:This research was supported by a grant ( 2010-0009233 ) from NRF , an Ewha W. University grant ( 2006-0114-1-1 ), a grant ( 2011-0006244 ) from NCRC program and a grant ( A080663 ) from KHIDI, the Republic of Korea .
Keywords
- Complex I
- DJ-1
- Dopaminergic neurons
- HSP60
- Oxidative stress
- PINK1