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

Jung Hee Shim, Seung Hee Yoon, Kyung Hee Kim, Ji Young Han, Ji Young Ha, Dong Hoon Hyun, Sun Ha Paek, Un Jung Kang, Xiaoxi Zhuang, Jin H. Son

Research output: Contribution to journalArticlepeer-review

36 Scopus citations

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 languageEnglish
Pages (from-to)707-715
Number of pages9
JournalMitochondrion
Volume11
Issue number5
DOIs
StatePublished - Sep 2011

Keywords

  • Complex I
  • DJ-1
  • Dopaminergic neurons
  • HSP60
  • Oxidative stress
  • PINK1

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