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Human Molecular Genetics Advance Access published online on March 18, 2009
Human Molecular Genetics, doi:10.1093/hmg/ddp093
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© The Author 2009. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

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

Sarika Srivastava1,§,£, Francisca Diaz1,§, Luisa Iommarini1,2, Karine Aure3, Anne Lombes3 and Carlos T. Moraes1,4,*

1 Department of Neurology, University of Miami School of Medicine, Miami, FL 33136, USA 2 Dipartimento di Scienze Neurologiche, Università di Bologna, Bologna, Italy 3 Inserm 582; UPMC-Paris6; AP/HP, Paris, F-75013 France 4 Department of Cell Biology and Anatomy, University of Miami School of Medicine, Miami, FL 33136, USA

* To whom correspondence should be addressed at: Department of Neurology, University of Miami School of Medicine, 1095 NW 14th Terrace, Miami, FL 33136, USA. Tel: +1 305 243 5858; Fax: +1 305 243 3914; Email: cmoraes{at}med.miami.edu

Received August 20, 2008; Revised February 3, 2009; Accepted February 24, 2009

Members of the peroxisome proliferator activated receptor {gamma} 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 (OXPHOS) defects due to either nuclear or mitochondrial DNA mutations. We found that the PGC-1{alpha} and/or PGC-1β expression improved mitochondrial respiration in cells harboring a complex III or a complex IV deficiency as well as in transmitochondrial cybrids harboring MELAS 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.

£ Current address, Harvard Medical School, Boston, MN 02115, USA

§ The first two authors contributed equally to this work


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