DJ-1 binds to mitochondrial complex I and maintains its activity.
Parkinson's disease (PD) is caused by neuronal cell death, and oxidative stress and mitochondrial dysfunction are thought to be responsible for onset of PD. DJ-1, a causative gene product of a familial form of Parkinson's disease, PARK7, plays roles in transcriptional regulation and anti-oxidative stress. The possible mitochondrial function of DJ-1 has been proposed, but its exact function remains unclear. In this study, we found that DJ-1 directly bound to NDUFA4 and ND1, nuclear and mitochondrial DNA-encoding subunits of mitochondrial complex I, respectively, and was colocalized with complex I and that complex I activity was reduced in DJ-1-knockdown NIH3T3 and HEK293 cells. These findings suggest that DJ-1 is an integral mitochondrial protein and that DJ-1 plays a role in maintenance of mitochondrial complex I activity.
Hayashi
Takuya
T
Graduate School of Agriculture, Hokkaido University, Sapporo 060-8589, Japan.
Ishimori
Chikako
C
Takahashi-Niki
Kazuko
K
Taira
Takahiro
T
Kim
Yun-chul
YC
Maita
Hiroshi
H
Maita
Chinatsu
C
Ariga
Hiroyoshi
H
Iguchi-Ariga
Sanae M M
SM
D000818:Animals; D042967:Electron Transport Complex I / Q000378:metabolism*; D003576:Electron Transport Complex IV / Q000235:genetics / Q000378:metabolism*; D055785:Gene Knockdown Techniques; D006801:Humans; D047908:Intracellular Signaling Peptides and Proteins / Q000235:genetics / Q000378:metabolism*; D051379:Mice; D015513:Oncogene Proteins / Q000235:genetics / Q000378:metabolism*; D010300:Parkinson Disease / Q000201:enzymology*; D011485:Protein Binding; D000071617:Protein Deglycase DJ-1
D047908:Intracellular Signaling Peptides and Proteins; D015513:Oncogene Proteins; D003576:Electron Transport Complex IV; C577147:NDUFA4 protein, human; C105131:PARK7 protein, human; D000071617:Protein Deglycase DJ-1; D042967:Electron Transport Complex I