Respiratory chain inhibition: one more feature to propose MPTP intoxication as a Leigh syndrome model

J Bioenerg Biomembr. 2016 Oct;48(5):483-491. doi: 10.1007/s10863-016-9683-7. Epub 2016 Oct 27.

Abstract

1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) intoxicated mice have been widely used to model the loss of dopaminergic neurons. As this treatment leads to basal ganglia degeneration, it was proposed that MPTP mice could be used as a model of Leigh syndrome. However, this mitochondrial pathology is biochemically characterized by a respiratory chain dysfunction. To determine if MPTP can affect in vivo mitochondria function, we measured the activities of mitochondrial respiratory chain complexes in several tissues. Our results show that MPTP affects mainly mitochondrial respiratory chain complex IV, as found in Leigh Syndrome, confirming that acute MPTP intoxicated mice are a good model of Leigh Syndrome.

Keywords: MPTP; Mice model; Mitochondrial diseases; Respiratory chain.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine / administration & dosage
  • 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine / adverse effects*
  • Animals
  • Disease Models, Animal*
  • Electron Transport / drug effects*
  • Electron Transport Complex IV / drug effects
  • Leigh Disease / chemically induced*
  • MPTP Poisoning
  • Mice
  • Mitochondria / metabolism

Substances

  • 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine
  • Electron Transport Complex IV