Detailed analysis of mitochondrial respiratory chain defects caused by loss of PINK1

Neurosci Lett. 2014 Sep 19:580:37-40. doi: 10.1016/j.neulet.2014.07.045. Epub 2014 Aug 1.


Mutations in PTEN-induced putative kinase 1 (PINK1) cause recessive forms of Parkinson's disease (PD). PINK1 acts upstream of parkin, regulating mitochondrial elimination (mitophagy) in cultured cells treated with mitochondrial uncouplers that cause mitochondrial depolarization. PINK1 loss-of-function decreases mitochondrial membrane potential, resulting in mitochondrial dysfunction, although the exact function of PINK1 in mitochondria has not been fully elucidated. We have previously found that PINK1 deficiency causes a decrease in mitochondrial membrane potential, which is not due to a proton leak, but to respiratory chain defects. Here, we examine mitochondrial respiratory chain defects in PINK1-deficient cells, and find both complex I and complex III are defective. These results suggest that mitochondrial respiratory chain defects may be associated with PD pathogenesis caused by mutations in the PINK1 gene.

Keywords: Mitochondria; PINK1; Parkinson's disease; Respiratory chain.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Cells, Cultured
  • Electron Transport
  • Electron Transport Chain Complex Proteins / metabolism
  • Fibroblasts / metabolism
  • Mice, Knockout
  • Mitochondria / metabolism*
  • Protein Kinases / metabolism*
  • Voltage-Dependent Anion Channels / metabolism


  • Electron Transport Chain Complex Proteins
  • Voltage-Dependent Anion Channels
  • Protein Kinases
  • PTEN-induced putative kinase