title: Idebenone increases mitochondrial complex I activity in fibroblasts from LHON patients while producing contradictory effects on respiration.
abstract: BACKGROUND
  Leber's hereditary optic neuropathy (LHON) is caused by mutations in the complex I subunits of the respiratory chain. Although patients have been treated with idebenone since 1992, the efficacy of the drug is still a matter of debate.
  
  
  METHODS
  We evaluated the effect of idebenone in fibroblasts from LHON patients using enzymatic and polarographic measurements.
  
  
  RESULTS
  Complex I activity was 42% greater in treated fibroblasts compared to controls (p = 0.002). Despite this complex I activity improvement, the effects on mitochondrial respiration were contradictory, leading to impairment in some cases and stimulation in others.
  
  
  CONCLUSIONS
  These results indicate that idebenone is able to compensate the complex I deficiency in LHON patient cells with variable effects on respiration, indicating that the patients might not be equally likely to benefit from the treatment.
authors.affiliation: INSERM U771, Angers 49000, France. pareynier@chu-angers.fr.
authors.forename: Claire
authors.initials: C
authors.lastname: Angebault
authors.forename: Naïg
authors.initials: N
authors.lastname: Gueguen
authors.forename: Valérie
authors.initials: V
authors.lastname: Desquiret-Dumas
authors.forename: Arnaud
authors.initials: A
authors.lastname: Chevrollier
authors.forename: Virginie
authors.initials: V
authors.lastname: Guillet
authors.forename: Christophe
authors.initials: C
authors.lastname: Verny
authors.forename: Julien
authors.initials: J
authors.lastname: Cassereau
authors.forename: Marc
authors.initials: M
authors.lastname: Ferre
authors.forename: Dan
authors.initials: D
authors.lastname: Milea
authors.forename: Patrizia
authors.initials: P
authors.lastname: Amati-Bonneau
authors.forename: Dominique
authors.initials: D
authors.lastname: Bonneau
authors.forename: Vincent
authors.initials: V
authors.lastname: Procaccio
authors.forename: Pascal
authors.initials: P
authors.lastname: Reynier
authors.forename: Dominique
authors.initials: D
authors.lastname: Loiseau