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 language | English |
|---|---|
| Pages (from-to) | 1805-1812 |
| Number of pages | 8 |
| Journal | Human Molecular Genetics |
| Volume | 18 |
| Issue number | 10 |
| DOIs | |
| State | Published - May 7 2009 |
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ASJC Scopus subject areas
- Genetics
- Genetics(clinical)
- Molecular Biology
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PGC-1α/β induced expression partially compensates for respiratory chain defects in cells from patients with mitochondrial disorders. / Srivastava, Sarika; Diaz, Francisca; Iommarini, Luisa; Aure, Karine; Lombes, Anne; Moraes, Carlos T.
In: Human Molecular Genetics, Vol. 18, No. 10, 07.05.2009, p. 1805-1812.Research output: Contribution to journal › Article
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TY - JOUR
T1 - PGC-1α/β induced expression partially compensates for respiratory chain defects in cells from patients with mitochondrial disorders
AU - Srivastava, Sarika
AU - Diaz, Francisca
AU - Iommarini, Luisa
AU - Aure, Karine
AU - Lombes, Anne
AU - Moraes, Carlos T
PY - 2009/5/7
Y1 - 2009/5/7
N2 - 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.
AB - 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.
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UR - http://www.scopus.com/inward/citedby.url?scp=65449133905&partnerID=8YFLogxK
U2 - 10.1093/hmg/ddp093
DO - 10.1093/hmg/ddp093
M3 - Article
C2 - 19297390
AN - SCOPUS:65449133905
VL - 18
SP - 1805
EP - 1812
JO - Human Molecular Genetics
JF - Human Molecular Genetics
SN - 0964-6906
IS - 10
ER -