PGC-1α Deficiency Causes Multi-System Energy Metabolic Derangements: Muscle Dysfunction, Abnormal Weight Control and Hepatic Steatosis

@article{Leone2005PGC1DC,
  title={PGC-1$\alpha$ Deficiency Causes Multi-System Energy Metabolic Derangements: Muscle Dysfunction, Abnormal Weight Control and Hepatic Steatosis},
  author={T. Leone and J. J. Lehman and B. Finck and P. Schaeffer and A. R. Wende and S. Boudina and M. Courtois and D. Wozniak and N. Sambandam and C. Bernal-Mizrachi and Z. Chen and John O. Holloszy and D. Medeiros and R. Schmidt and J. Saffitz and E. Abel and C. Semenkovich and D. Kelly},
  journal={PLoS Biology},
  year={2005},
  volume={3}
}
The gene encoding the transcriptional coactivator peroxisome proliferator-activated receptor-γ coactivator-1α (PGC-1α) was targeted in mice. PGC-1α null (PGC-1α−/−) mice were viable. However, extensive phenotyping revealed multi-system abnormalities indicative of an abnormal energy metabolic phenotype. The postnatal growth of heart and slow-twitch skeletal muscle, organs with high mitochondrial energy demands, is blunted in PGC-1α−/− mice. With age, the PGC-1α−/− mice develop abnormally… Expand
Total skeletal muscle PGC-1 deficiency uncouples mitochondrial derangements from fiber type determination and insulin sensitivity.
The transcriptional coactivator PGC-1alpha is essential for maximal and efficient cardiac mitochondrial fatty acid oxidation and lipid homeostasis.
A Role for Peroxisome Proliferator-activated Receptor γ Coactivator 1 (PGC-1) in the Regulation of Cardiac Mitochondrial Phospholipid Biosynthesis*♦
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