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. 2004 Oct 1;119(1):121-35.
doi: 10.1016/j.cell.2004.09.013.

Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1alpha null mice

Jiandie Lin  1 , Pei-Hsuan Wu, Paul T Tarr, Katrin S Lindenberg, Julie St-Pierre, Chen-Yu Zhang, Vamsi K Mootha, Sibylle Jäger, Claudia R Vianna, Richard M Reznick, Libin Cui, Monia Manieri, Mi X Donovan, Zhidan Wu, Marcus P Cooper, Melina C Fan, Lindsay M Rohas, Ann Marie Zavacki, Saverio Cinti, Gerald I Shulman, Bradford B Lowell, Dimitri Krainc, Bruce M Spiegelman
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Free article

Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1alpha null mice

Jiandie Lin et al. Cell. .
Free article

Abstract

PGC-1alpha is a coactivator of nuclear receptors and other transcription factors that regulates several metabolic processes, including mitochondrial biogenesis and respiration, hepatic gluconeogenesis, and muscle fiber-type switching. We show here that, while hepatocytes lacking PGC-1alpha are defective in the program of hormone-stimulated gluconeogenesis, the mice have constitutively activated gluconeogenic gene expression that is completely insensitive to normal feeding controls. C/EBPbeta is elevated in the livers of these mice and activates the gluconeogenic genes in a PGC-1alpha-independent manner. Despite having reduced mitochondrial function, PGC-1alpha null mice are paradoxically lean and resistant to diet-induced obesity. This is largely due to a profound hyperactivity displayed by the null animals and is associated with lesions in the striatal region of the brain that controls movement. These data illustrate a central role for PGC-1alpha in the control of energy metabolism but also reveal novel systemic compensatory mechanisms and pathogenic effects of impaired energy homeostasis.

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