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The antioxidant Trolox helps recovery from the familial Parkinson's disease-specific mitochondrial deficits caused by PINK1- and DJ-1-deficiency in dopaminergic neuronal cells

Title
The antioxidant Trolox helps recovery from the familial Parkinson's disease-specific mitochondrial deficits caused by PINK1- and DJ-1-deficiency in dopaminergic neuronal cells
Authors
Shim J.H.Yoon S.H.Kim K.-H.Han J.Y.Ha J.-Y.Hyun D.H.Paek S.H.Kang U.J.Zhuang X.Son J.H.
Ewha Authors
손형진현동훈
SCOPUS Author ID
손형진scopus; 현동훈scopus
Issue Date
2011
Journal Title
Mitochondrion
ISSN
1567-7249JCR Link
Citation
vol. 11, no. 5, pp. 707 - 715
Indexed
SCIE; SCOPUS WOS scopus
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. © 2011.
DOI
10.1016/j.mito.2011.05.013
Appears in Collections:
약학대학 > 약학과 > Journal papers
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