Journal Article

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 and Carlos T. Moraes

in Human Molecular Genetics

Volume 18, issue 10, pages 1805-1812
Published in print May 2009 | ISSN: 0964-6906
Published online March 2009 | e-ISSN: 1460-2083 | DOI: http://dx.doi.org/10.1093/hmg/ddp093
PGC-1α/β induced expression partially compensates for respiratory chain defects in cells from patients with mitochondrial disorders

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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.

Journal Article.  4207 words.  Illustrated.

Subjects: Genetics and Genomics

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