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Myopathy caused by mammalian target of rapamycin complex 1 (mTORC1) inactivation is not reversed by restoring mitochondrial function.

Romanino K, Mazelin L, Albert V, Conjard-Duplany A, Lin S, Bentzinger CF, Handschin C, Puigserver P, Zorzato F, Schaeffer L, Gangloff YG, Rüegg MA.

Proc Natl Acad Sci U S A. 2011 Dec 20;108(51):20808-13. doi: 10.1073/pnas.1111448109. Epub 2011 Dec 5.


Skeletal muscle-specific expression of PGC-1α-b, an exercise-responsive isoform, increases exercise capacity and peak oxygen uptake.

Tadaishi M, Miura S, Kai Y, Kano Y, Oishi Y, Ezaki O.

PLoS One. 2011;6(12):e28290. doi: 10.1371/journal.pone.0028290. Epub 2011 Dec 8.


A role for the transcriptional coactivator PGC-1alpha in muscle refueling.

Wende AR, Schaeffer PJ, Parker GJ, Zechner C, Han DH, Chen MM, Hancock CR, Lehman JJ, Huss JM, McClain DA, Holloszy JO, Kelly DP.

J Biol Chem. 2007 Dec 14;282(50):36642-51. Epub 2007 Oct 11.


The role of PGC-1alpha on mitochondrial function and apoptotic susceptibility in muscle.

Adhihetty PJ, Uguccioni G, Leick L, Hidalgo J, Pilegaard H, Hood DA.

Am J Physiol Cell Physiol. 2009 Jul;297(1):C217-25. doi: 10.1152/ajpcell.00070.2009. Epub 2009 May 13.


PGC-1{alpha} is required for AICAR-induced expression of GLUT4 and mitochondrial proteins in mouse skeletal muscle.

Leick L, Fentz J, Biensø RS, Knudsen JG, Jeppesen J, Kiens B, Wojtaszewski JF, Pilegaard H.

Am J Physiol Endocrinol Metab. 2010 Sep;299(3):E456-65. doi: 10.1152/ajpendo.00648.2009. Epub 2010 Jul 13.


Differential response of skeletal muscles to mTORC1 signaling during atrophy and hypertrophy.

Bentzinger CF, Lin S, Romanino K, Castets P, Guridi M, Summermatter S, Handschin C, Tintignac LA, Hall MN, Rüegg MA.

Skelet Muscle. 2013 Mar 6;3(1):6. doi: 10.1186/2044-5040-3-6.


Skeletal muscle-specific ablation of raptor, but not of rictor, causes metabolic changes and results in muscle dystrophy.

Bentzinger CF, Romanino K, Cloëtta D, Lin S, Mascarenhas JB, Oliveri F, Xia J, Casanova E, Costa CF, Brink M, Zorzato F, Hall MN, Rüegg MA.

Cell Metab. 2008 Nov;8(5):411-24. doi: 10.1016/j.cmet.2008.10.002.


PGC-1α induces mitochondrial and myokine transcriptional programs and lipid droplet and glycogen accumulation in cultured human skeletal muscle cells.

Mormeneo E, Jimenez-Mallebrera C, Palomer X, De Nigris V, Vázquez-Carrera M, Orozco A, Nascimento A, Colomer J, Lerín C, Gómez-Foix AM.

PLoS One. 2012;7(1):e29985. doi: 10.1371/journal.pone.0029985. Epub 2012 Jan 17.


Activation of the PPAR/PGC-1alpha pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype.

Wenz T, Diaz F, Spiegelman BM, Moraes CT.

Cell Metab. 2008 Sep;8(3):249-56. doi: 10.1016/j.cmet.2008.07.006.


Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington's disease.

Johri A, Calingasan NY, Hennessey TM, Sharma A, Yang L, Wille E, Chandra A, Beal MF.

Hum Mol Genet. 2012 Mar 1;21(5):1124-37. doi: 10.1093/hmg/ddr541. Epub 2011 Nov 17.


mTOR controls mitochondrial oxidative function through a YY1-PGC-1alpha transcriptional complex.

Cunningham JT, Rodgers JT, Arlow DH, Vazquez F, Mootha VK, Puigserver P.

Nature. 2007 Nov 29;450(7170):736-40.


PGC-1alpha increases PDH content but does not change acute PDH regulation in mouse skeletal muscle.

Kiilerich K, Adser H, Jakobsen AH, Pedersen PA, Hardie DG, Wojtaszewski JF, Pilegaard H.

Am J Physiol Regul Integr Comp Physiol. 2010 Nov;299(5):R1350-9. doi: 10.1152/ajpregu.00400.2010. Epub 2010 Aug 18.


PGC-1alpha integrates insulin signaling, mitochondrial regulation, and bioenergetic function in skeletal muscle.

Pagel-Langenickel I, Bao J, Joseph JJ, Schwartz DR, Mantell BS, Xu X, Raghavachari N, Sack MN.

J Biol Chem. 2008 Aug 15;283(33):22464-72. doi: 10.1074/jbc.M800842200. Epub 2008 Jun 25.


Adiponectin increases skeletal muscle mitochondrial biogenesis by suppressing mitogen-activated protein kinase phosphatase-1.

Qiao L, Kinney B, Yoo HS, Lee B, Schaack J, Shao J.

Diabetes. 2012 Jun;61(6):1463-70. doi: 10.2337/db11-1475. Epub 2012 Mar 13.


Effect of bezafibrate treatment on late-onset mitochondrial myopathy in mice.

Yatsuga S, Suomalainen A.

Hum Mol Genet. 2012 Feb 1;21(3):526-35. doi: 10.1093/hmg/ddr482. Epub 2011 Oct 19.


Contractile activity-induced mitochondrial biogenesis and mTORC1.

Carter HN, Hood DA.

Am J Physiol Cell Physiol. 2012 Sep 1;303(5):C540-7. doi: 10.1152/ajpcell.00156.2012. Epub 2012 Jun 13.


Mitochondrial biogenesis and PGC-1α deacetylation by chronic treadmill exercise: differential response in cardiac and skeletal muscle.

Li L, Mühlfeld C, Niemann B, Pan R, Li R, Hilfiker-Kleiner D, Chen Y, Rohrbach S.

Basic Res Cardiol. 2011 Nov;106(6):1221-34. doi: 10.1007/s00395-011-0213-9. Epub 2011 Aug 28.


Mitochondrial biogenesis and peroxisome proliferator-activated receptor-γ coactivator-1α (PGC-1α) deacetylation by physical activity: intact adipocytokine signaling is required.

Li L, Pan R, Li R, Niemann B, Aurich AC, Chen Y, Rohrbach S.

Diabetes. 2011 Jan;60(1):157-67. doi: 10.2337/db10-0331. Epub 2010 Oct 7.


PGC-1alpha is coupled to HIF-1alpha-dependent gene expression by increasing mitochondrial oxygen consumption in skeletal muscle cells.

O'Hagan KA, Cocchiglia S, Zhdanov AV, Tambuwala MM, Cummins EP, Monfared M, Agbor TA, Garvey JF, Papkovsky DB, Taylor CT, Allan BB.

Proc Natl Acad Sci U S A. 2009 Feb 17;106(7):2188-93. doi: 10.1073/pnas.0808801106. Epub 2009 Jan 28. Erratum in: Proc Natl Acad Sci U S A. 2009 Mar 31;106(13):5449. Tambawala, Murtaza M [corrected to Tambuwala, Murtaza M].


Carbohydrate metabolism is perturbed in peroxisome-deficient hepatocytes due to mitochondrial dysfunction, AMP-activated protein kinase (AMPK) activation, and peroxisome proliferator-activated receptor γ coactivator 1α (PGC-1α) suppression.

Peeters A, Fraisl P, van den Berg S, Ver Loren van Themaat E, Van Kampen A, Rider MH, Takemori H, van Dijk KW, Van Veldhoven PP, Carmeliet P, Baes M.

J Biol Chem. 2011 Dec 9;286(49):42162-79. doi: 10.1074/jbc.M111.299727. Epub 2011 Oct 14.

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