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

Authors: Lin, Jiandie  Wu, Pei-Hsuan  Tarr, Paul T  Lindenberg, Katrin S  St-Pierre, Julie  Zhang, Chen-Yu  Mootha, Vamsi K  J├Ąger, Sibylle  Vianna, Claudia R  Reznick, Richard M  Cui, Libin  Manieri, Monia  Donovan, Mi X  Wu, Zhidan  Cooper, Marcus P  Fan, Melina C  Rohas, Lindsay M  Zavacki, Ann Marie  Cinti, Saverio  Shulman, Gerald I  Lowell, Bradford B  Krainc, Dimitri  Spiegelman, Bruce M 
Citation: Lin J, etal., Cell. 2004 Oct 1;119(1):121-35. doi: 10.1016/j.cell.2004.09.013.
Pubmed: (View Article at PubMed) PMID:15454086
DOI: Full-text: DOI:10.1016/j.cell.2004.09.013

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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RGD Object Information
RGD ID: 13673838
Created: 2018-06-23
Species: All species
Last Modified: 2018-06-23
Status: ACTIVE


RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.