Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration.

By P. Weydt, Victor V. Pineda, A. E. Torrence, R. Libby, Terrence F. Satterfield, E. Lazarowski, M. Gilbert, G. Morton, T. Bammler, A. Strand and 9 more. Cell Metabolism, 2006

605 citations recorded by the source corpus.

Subfields: Cellular and Molecular Neuroscience, Molecular Biology, Physiology

View Paper: https://www.semanticscholar.org/paper/d1ffc6503e2546f1a30dbcbc06c94a4cbff00b2e

How researchers see this paper

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In Cellular and Molecular Neuroscience

1. Mark P. Mattson
2. D. Holtzman
3. Hilmar Bading
4. G. Hardingham
5. M. Beal
6. A. Johri
7. L. Zuo
8. Wenzhen Duan

In Molecular Biology

1. A. Johri
2. M. Beal
3. Christopher A. Ross
4. Hilmar Bading
5. Mark P. Mattson
6. G. Hardingham
7. D. Holtzman
8. D. Krainc

In Physiology

1. M. Beal
2. L. Zuo
3. Christopher A. Ross
4. Zewen Liu
5. P. Dimitrion
6. Mark P. Mattson
7. A. Johri
8. D. Holtzman

Citations in the corpus

• Synaptic versus extrasynaptic NMDA receptor signalling: implications for neurodegenerative disorders (2010, positive): “MtHTT interferes with CREB-dependent PGC1α expression, and this leads to mitochondrial dysfunction and metabolic defect”
• Oxidative Stress in Neurodegenerative Diseases: From Molecular Mechanisms to Clinical Applications (2017, background): “mHTT has been shown to reduce the expression of peroxisome proliferator-activated receptor-γ coactivator-1α, altering the level of antioxidant enzymes and lowering the concentration of striatal mitochondria [66, 70, 71].”
• Mitochondrial Dysfunction in Neurodegenerative Diseases (2012, background): “…reduce A␤ plaque in an in vitro model of AD, produce neuroprotective effects in a transgenic mouse model of ALS, and enhance the mitochondrial membrane potential and reduce mitochondrial toxicity in in vitro models of HD (Weydt et al., 2006; Qin et al., 2009; Liang et al., 2011; Zhao et al., 2011).”
• Neuroprotective role of SIRT1 in mammalian models of Huntington’s disease through activation of multiple SIRT1 targets (2011, positive): “PGC-1 α is also a substrate of Sirt1 and has been previously implicated in Huntington’s disease pathogenesis 36–38 .”