First Author | Shim JH | Year | 2011 |
Journal | Mitochondrion | Volume | 11 |
Issue | 5 | Pages | 707-15 |
PubMed ID | 21664494 | Mgi Jnum | J:192145 |
Mgi Id | MGI:5464085 | Doi | 10.1016/j.mito.2011.05.013 |
Citation | Shim JH, et al. (2011) 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. Mitochondrion 11(5):707-15 |
abstractText | 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. |