PGC-1α/β induced expression partially compensates for respiratory chain defects in cells from patients with mitochondrial disorders

Sarika Srivastava, Francisca Diaz, Luisa Iommarini, Karine Aure, Anne Lombes, Carlos T. Moraes

Research output: Contribution to journalArticle

73 Scopus citations

Abstract

Members of the peroxisome proliferator-activated receptor γ coactivator (PGC) family are potent inducers of mitochondrial biogenesis. We have tested the potential effect of increased mitochondrial biogenesis in cells derived from patients harboring oxidative phosphorylation defects due to either nuclear or mitochondrial DNA mutations. We found that the PGC-1α and/or PGC-1β expression improved mitochondrial respiration in cells harboring a complex III or IV deficiency as well as in transmitochondrial cybrids harboring mitochondrial encephalomyopathy lactic acidosis and stroke A3243G tRNA(Leu)UUR gene mutation. The respiratory function improvement was found to be associated with increased levels of mitochondrial components per cell, although this increase was not homogeneous. These results reinforce the concept that increased mitochondrial biogenesis is a promising venue for the treatment of mitochondrial diseases.

Original languageEnglish (US)
Pages (from-to)1805-1812
Number of pages8
JournalHuman molecular genetics
Volume18
Issue number10
DOIs
StatePublished - 2009

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics
  • Genetics(clinical)

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