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

Bioconductor: open software development for computational biology and bioinformatics

Gentleman, R., Carey, V., Bates, D., Bolstad, B., Dettling, M., Dudoit, S., Ellis, B., Gautier, L., Ge, Y., Gentry, J., et al. (2004) · Genome Biology · doi:10.1186/gb-2004-5-10-r80 · Cited by 13,133 · see citing works

PGC-1α-responsive genes involved in oxidative phosphorylation are coordinately downregulated in human diabetes

Mootha, V. K., Lindgren, C. M., Eriksson, K.-F., Subramanian, A., Sihag, S., Lehar, J., Puigserver, P., Carlsson, E., Ridderstråle, M., Laurila, E., et al. (2003) · Nature Genetics · doi:10.1038/ng1180 · Cited by 10,197 · see citing works

Statistical significance for genomewide studies

Storey, J. D., & Tibshirani, R. (2003) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1530509100 · Cited by 9,692 · see citing works

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes

MACDONALD, M. (1993) · Cell · doi:10.1016/0092-8674(93)90585-e · Cited by 8,366 · see citing works

A comparison of normalization methods for high density oligonucleotide array data based on variance and bias

Bolstad, B. M., Irizarry, R. A., Åstrand, M., & Speed, T. P. (2003) · Bioinformatics · doi:10.1093/bioinformatics/19.2.185 · Cited by 8,185 · see citing works

Brown Adipose Tissue: Function and Physiological Significance

CANNON, B., & NEDERGAARD, J. (2004) · Physiological Reviews · doi:10.1152/physrev.00015.2003 · Cited by 6,433 · see citing works

Motor Neuron Degeneration in Mice that Express a Human Cu,Zn Superoxide Dismutase Mutation

Gurney, M. E., Pu, H., Chiu, A. Y., Dal Canto, M. C., Polchow, C. Y., Alexander, D. D., Caliendo, J., Hentati, A., Kwon, Y. W., Deng, H.-X., et al. (1994) · Science · doi:10.1126/science.8209258 · Cited by 4,143 · see citing works

A Cold-Inducible Coactivator of Nuclear Receptors Linked to Adaptive Thermogenesis

Puigserver, P., Wu, Z., Park, C. W., Graves, R., Wright, M., & Spiegelman, B. M. (1998) · Cell · doi:10.1016/s0092-8674(00)81410-5 · Cited by 3,864 · see citing works

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

Mangiarini, L., Sathasivam, K., Seller, M., Cozens, B., Harper, A., Hetherington, C., Lawton, M., Trottier, Y., Lehrach, H., Davies, S. W., et al. (1996) · Cell · doi:10.1016/s0092-8674(00)81369-0 · Cited by 3,073 · see citing works

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group.

Shah, M. A., Datson, N. A., Srinidhi, L., Stanton, V. P., MacDonald, M. E., Allard, M. W., Youngman, S., Am, F., Mott, R., Draths, K., et al. (1993) · Cell · Cited by 3,055 · see citing works

Metabolic control through the PGC-1 family of transcription coactivators

Lin, J., Handschin, C., & Spiegelman, B. M. (2005) · Cell Metabolism · doi:10.1016/j.cmet.2005.05.004 · Cited by 2,130 · see citing works

Peroxisome Proliferator-Activated Receptor-γ Coactivator 1α (PGC-1α): Transcriptional Coactivator and Metabolic Regulator

Puigserver, P., & Spiegelman, B. M. (2003) · Endocrine Reviews · doi:10.1210/er.2002-0012 · Cited by 2,076 · see citing works

Mitochondrial Respiratory-Chain Diseases

DiMauro, S., & Schon, E. A. (2003) · New England Journal of Medicine · doi:10.1056/nejmra022567 · Cited by 1,650 · see citing works

Towards a molecular understanding of adaptive thermogenesis

Lowell, B. B., & Spiegelman, B. M. (2000) · Nature · doi:10.1038/35007527 · Cited by 1,630 · see citing works

Oligonucleotides with fluorescent dyes at opposite ends provide a quenched probe system useful for detecting PCR product and nucleic acid hybridization.Preprint

Livak, K. J., Flood, S., Marmaro, J., Giusti, W. G., & Deetz, K. (1995) · Genome Research · doi:10.1101/gr.4.6.357 · Cited by 1,602 · see citing works

Use of within-array replicate spots for assessing differential expression in microarray experiments

Smyth, G. K., Michaud, J., & Scott, H. S. (2005) · Computer applications in the biosciences · doi:10.1093/bioinformatics/bti270 · Cited by 1,406 · see citing works

Glutamine Repeats and Neurodegeneration

Zoghbi, H. Y., & Orr, H. T. (2000) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.23.1.217 · Cited by 1,311 · see citing works

The Coactivator PGC-1 Cooperates with Peroxisome Proliferator-Activated Receptor α in Transcriptional Control of Nuclear Genes Encoding Mitochondrial Fatty Acid Oxidation Enzymes

Vega, R. B., Huss, J. M., & Kelly, D. P. (2000) · Molecular and Cellular Biology · doi:10.1128/mcb.20.5.1868-1876.2000 · Cited by 1,195 · see citing works

Defects in Adaptive Energy Metabolism with CNS-Linked Hyperactivity in PGC-1α Null Mice

Lin, J., Wu, P.-H., Tarr, P. T., Lindenberg, K. S., St-Pierre, J., Zhang, C.-Y., Mootha, V. K., Jäger, S., Vianna, C. R., Reznick, R. M., et al. (2004) · Cell · doi:10.1016/j.cell.2004.09.013 · Cited by 1,162 · see citing works

Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid

Beal, M. F., Brouillet, E., Jenkins, B. G., Ferrante, R. J., Kowall, N. W., Miller, J., Storey, E., Srivastava, R., Rosen, B. R., & Hyman, B. T. (1993) · Journal of Neuroscience · doi:10.1523/jneurosci.13-10-04181.1993 · Cited by 1,026 · see citing works

Transcriptional Repression of PGC-1α by Mutant Huntingtin Leads to Mitochondrial Dysfunction and Neurodegeneration

Cui, L., Jeong, H., Borovecki, F., Parkhurst, C. N., Tanese, N., & Krainc, D. (2006) · Cell · doi:10.1016/j.cell.2006.09.015 · Cited by 1,012 · see citing works

Mitochondria take center stage in aging and neurodegeneration

Beal, M. F. (2005) · Annals of Neurology · doi:10.1002/ana.20624 · Cited by 957 · see citing works

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

Leone, T. C., Lehman, J. J., Finck, B. N., Schaeffer, P. J., Wende, A. R., Boudina, S., Courtois, M., Wozniak, D. F., Sambandam, N., Bernal-Mizrachi, C., et al. (2005) · PLoS Biology · doi:10.1371/journal.pbio.0030101 · Cited by 951 · see citing works

Oxidative damage and metabolic dysfunction in Huntington's disease: Selective vulnerability of the basal ganglia

Browne, S. E., Bowling, A. C., Macgarvey, U., Baik, M. J., Berger, S. C., Muquit, M. M. K., Bird, E. D., & Beal, M. F. (1997) · Annals of Neurology · doi:10.1002/ana.410410514 · Cited by 885 · see citing works

A YAC Mouse Model for Huntington’s Disease with Full-Length Mutant Huntingtin, Cytoplasmic Toxicity, and Selective Striatal Neurodegeneration

Hodgson, J., Agopyan, N., Gutekunst, C., Leavitt, B. R., LePiane, F. G., Singaraja, R. R., Smith, D. J., Bissada, N., McCutcheon, K. M., Nasir, J., et al. (1999) · Neuron · doi:10.1016/s0896-6273(00)80764-3 · Cited by 832 · see citing works

Regional and cellular gene expression changes in human Huntington's disease brain

Hodges, A., Strand, A. D., Aragaki, A. K., Kuhn, A., Sengstag, T., Hughes, G., Elliston, L. A., Hartog, C., Goldstein, D. R., Thu, D., et al. (2006) · Human Molecular Genetics · doi:10.1093/hmg/ddl013 · Cited by 804 · see citing works

Mitochondrial defect in Huntington's disease caudate nucleus

Gu, M., Gash, M. T., Mann, V. M., Javoy‐Agid, F., Cooper, J. M., & Schapira, A. H. V. (1996) · Annals of Neurology · doi:10.1002/ana.410390317 · Cited by 758 · see citing works

Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin [published erratum appears in Hum Mol Genet 1999 May;8(5):943]

Schilling, G. (1999) · Human Molecular Genetics · doi:10.1093/hmg/8.3.397 · Cited by 755 · see citing works

Decreased expression of striatal signaling genes in a mouse model of Huntington's disease

Luthi‐Carter, R. (2000) · Human Molecular Genetics · doi:10.1093/hmg/9.9.1259 · Cited by 723 · see citing works

Dominant phenotypes produced by the HD mutation in STHdhQ111 striatal cells

Trettel, F. (2000) · Human Molecular Genetics · doi:10.1093/hmg/9.19.2799 · Cited by 614 · see citing works

A huntingtin-associated protein enriched in brain with implications for pathology

Li, X., Li, S.-H., Sharp, A. H., Nucifora, F. C., Schilling, G., Lanahan, A. A., Worley, P. F., Snyder, S. H., & Ross, C. A. (1995) · Nature · doi:10.1038/378398a0 · Cited by 605 · see citing works

Transcriptional abnormalities in Huntington disease

Sugars, K. L., & Rubinsztein, D. C. (2003) · Trends in Genetics · doi:10.1016/s0168-9525(03)00074-x · Cited by 529 · see citing works

Time course of early motor and neuropathological anomalies in a knock‐in mouse model of Huntington's disease with 140 CAG repeats

Menalled, L., Sison, J. D., Dragatsis, I., Zeitlin, S. O., & Chesselet, M. (2003) · The Journal of Comparative Neurology · doi:10.1002/cne.10776 · Cited by 528 · see citing works

Peroxisome proliferator‐activated receptor δ controls muscle development and oxydative capability

Luquet, S., Lopez‐Soriano, J., Holst, D., Fredenrich, A., Melki, J., Rassoulzadegan, M., & Grimaldi, P. A. (2003) · The FASEB Journal · doi:10.1096/fj.03-0269fje · Cited by 509 · see citing works

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders

Sánchez, I., Mahlke, C., & Yuan, J. (2003) · Nature · doi:10.1038/nature01301 · Cited by 504 · see citing works

Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates.

Brouillet, E., Hantraye, P., Ferrante, R. J., Dolan, R., Leroy‐Willig, A., Kowall, N. W., & Beal, M. F. (1995) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.92.15.7105 · Cited by 485 · see citing works

Frataxin Acts as an Iron Chaperone Protein to Modulate Mitochondrial Aconitase Activity

Bulteau, A.-L., O’Neill, H. A., Kennedy, M. C., Ikeda‐Saito, M., Isaya, G., & Szweda, L. I. (2004) · Science · doi:10.1126/science.1098991 · Cited by 372 · see citing works

HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism

Seong, I. S., Ivanova, E. V., Lee, J., Choo, Y. S., Fossale, E., Anderson, M., Gusella, J. F., Laramie, J. M., Myers, R. H., Lesort, M. J., et al. (2005) · Human Molecular Genetics · doi:10.1093/hmg/ddi319 · Cited by 291 · see citing works

Polyglutamine-Expanded Ataxin-7 Antagonizes CRX Function and Induces Cone-Rod Dystrophy in a Mouse Model of SCA7

Spada, A. R. L., Fu, Y., Sopher, B. L., Libby, R. T., Wang, X., Li, L. Y., Einum, D. D., Huang, J., Possin, D. E., Smith, A. C., et al. (2001) · Neuron · doi:10.1016/s0896-6273(01)00422-6 · Cited by 272 · see citing works

Nucleotide Release Provides a Mechanism for Airway Surface Liquid Homeostasis

Lazarowski, E. R., Tarran, R., Grubb, B. R., van Heusden, C. A., Okada, S. F., & Boucher, R. C. (2004) · Journal of Biological Chemistry · doi:10.1074/jbc.m405367200 · Cited by 236 · see citing works

Experimental therapeutics in transgenic mouse models of Huntington's disease

Beal, M. F., & Ferrante, R. J. (2004) · Nature reviews. Neuroscience · doi:10.1038/nrn1386 · Cited by 224 · see citing works

Active HSF1 Significantly Suppresses Polyglutamine Aggregate Formation in Cellular and Mouse Models

Fujimoto, M., Takaki, E., Hayashi, T., Kitaura, Y., Tanaka, Y., Inouye, S., & Nakai, A. (2005) · Journal of Biological Chemistry · doi:10.1074/jbc.m506288200 · Cited by 196 · see citing works

Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates

Brouillet, M. (1995) · Medical Entomology and Zoology · Cited by 190 · see citing works

Polyglutamine-Expanded Ataxin-7 Promotes Non-Cell-Autonomous Purkinje Cell Degeneration and Displays Proteolytic Cleavage in Ataxic Transgenic Mice

Garden, G. A., Libby, R. T., Fu, Y., Kinoshita, Y., Huang, J., Possin, D. E., Smith, A. C., Martinez, R. A., Fine, G. C., Grote, S. K., et al. (2002) · Journal of Neuroscience · doi:10.1523/jneurosci.22-12-04897.2002 · Cited by 167 · see citing works

The orphan nuclear receptor SHP regulates PGC-1α expression and energy production in brown adipocytes

Wang, L., Liu, J., Saha, P., Huang, J., Chan, L., Spiegelman, B. M., & Moore, D. D. (2005) · Cell Metabolism · doi:10.1016/j.cmet.2005.08.010 · Cited by 150 · see citing works

Lack of Huntingtin-Associated Protein-1 Causes Neuronal Death Resembling Hypothalamic Degeneration in Huntington's Disease

Li, S.-H., Yu, Z.-X., Li, C.-L., et al. (2003) · Journal of Neuroscience · doi:10.1523/jneurosci.23-17-06956.2003 · Cited by 132 · see citing works

Fasting and Leptin Modulate Adipose and Muscle Uncoupling Protein: Divergent Effects Between Messenger Ribonucleic Acid and Protein Expression1

Sivitz, W. I., Fink, B. D., & Donohoue, P. A. (1999) · Endocrinology · doi:10.1210/endo.140.4.6668 · Cited by 104 · see citing works

Bioenergetics in Huntington's Disease

Grünewald, T. G. P., & Beal, M. F. (1999) · Annals of the New York Academy of Sciences · doi:10.1111/j.1749-6632.1999.tb07827.x · Cited by 100 · see citing works

Continuous Measurement of Oxygen Consumption by Pancreatic Islets

Sweet, I., Khalil, G., Wallen, A., Steedman, M., Schenkman, K. A., Reems, J., Kahn, S., & Callis, J. (2002) · Diabetes Technology & Therapeutics · doi:10.1089/152091502320798303 · Cited by 100 · see citing works

Decreased N-acetyl-aspartate/choline ratio and increased lactate in the frontal lobe of patients with Huntington's disease: a proton magnetic resonance spectroscopy study.

Harms, L., Meierkord, H., Timm, G., Pfeiffer, L., & Ludolph, A. C. (1997) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.62.1.27 · Cited by 99 · see citing works

Regulation of ATP/ADP in pancreatic islets.

Sweet, I., Cook, D., DeJulio, E., Wallen, A., Khalil, G., Callis, J., & Reems, J. (2004) · Diabetes · doi:10.2337/diabetes.53.2.401 · Cited by 97 · see citing works

Regulatory Motifs for CREB-binding Protein and Nfe2l2 Transcription Factors in the Upstream Enhancer of the Mitochondrial Uncoupling Protein 1 Gene

Rim, J. S., & Kozak, L. P. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m108866200 · Cited by 95 · see citing works

UCP2, UCP3 and leptin gene expression: modulation by food restriction and leptin

Scarpace, P., Nicolson, M. A., & Matheny, M. E. (1998) · Journal of Endocrinology · doi:10.1677/joe.0.1590349 · Cited by 92 · see citing works

Hypothalamic huntingtin-associated protein 1 as a mediator of feeding behavior

Sheng, G., Chang, G., Lin, J. Y., Yu, Z.-X., Fang, Z.-H., Rong, J., Lipton, S. A., Li, S.-H., Tong, G., LEIBOWITZ, S. F., et al. (2006) · Nature Medicine · doi:10.1038/nm1382 · Cited by 90 · see citing works

Clinical Aspects of CAG Repeat Diseases

Nance, M. (1997) · Brain Pathology · doi:10.1111/j.1750-3639.1997.tb00892.x · Cited by 88 · see citing works

[18F]Fluorodeoxyglucose positron emission tomography in the diagnosis of cancer in patients with paraneoplastic neurological syndrome and anti-Hu antibodies

Antoine, J.-C. G., Cinotti, L., Tilikete, C., Bouhour, F., Camdessanch�, J. P., Confavreux, C., Vighetto, A., Renault‐Mannel, V., Michel, D., & Honnorat, J. (2000) · Annals of Neurology · doi:10.1002/1531-8249(200007)48:1 · Cited by 86 · see citing works

α1- and β1-Adrenoceptor Signaling Fully Compensates for β3-Adrenoceptor Deficiency in Brown Adipocyte Norepinephrine-Stimulated Glucose Uptake

Chernogubova, E., Hutchinson, D. S., Nedergaard, J., & Bengtsson, T. (2005) · Endocrinology · doi:10.1210/en.2004-1104 · Cited by 76 · see citing works

PET studies of cerebral glucose metabolism in idiopathic torticollis

Stoessl, A. J., Martin, W. R. W., Clark, C. C., et al. (1986) · Neurology · doi:10.1212/wnl.36.5.653 · Cited by 59 · see citing works

Weight changes in Huntington's disease

Robbins, A. O. G., Ho, A. K., & Barker, R. (2006) · European Journal of Neurology · doi:10.1111/j.1468-1331.2006.01319.x · Cited by 54 · see citing works

Meningeal cells can communicate with astrocytes by calcium signaling

Grafstein, B., Liu, S., Cotrina, M. L., Goldman, S. A., & Nedergaard, M. (2000) · Annals of Neurology · doi:10.1002/1531-8249(200001)47:1 · Cited by 50 · see citing works

Synaptic versus extrasynaptic NMDA receptor signalling: implications for neurodegenerative disorders

Hardingham, G. E., & Bading, H. (2010) · Nature Reviews Neuroscience · doi:10.1038/nrn2911 · Cited by 1,644 · see citing works

Mitochondria in neuroplasticity and neurological disorders

Mattson, M. P., Gleichmann, M., & Cheng, A. (2008) · Neuron · doi:10.1016/j.neuron.2008.10.010 · Cited by 1,081 · see citing works

Oxidative Stress in Neurodegenerative Diseases: From Molecular Mechanisms to Clinical Applications

Liu, Z., Zhou, T., Ziegler, A. C., Dimitrion, P., & Zuo, L. (2017) · Oxidative Medicine and Cellular Longevity · doi:10.1155/2017/2525967 · Cited by 810 · see citing works

Mitochondrial Dysfunction in Neurodegenerative Diseases

Johri, A., & Beal, M. F. (2012) · The Journal of Pharmacology and Experimental Therapeutics · doi:10.1124/jpet.112.192138 · Cited by 806 · see citing works

Sirtuin Functions in Health and Disease

Yamamoto, H., Schoonjans, K., & Auwerx, J. (2007) · Molecular Endocrinology · doi:10.1210/me.2007-0079 · Cited by 480 · see citing works

Huntington's disease: from pathology and genetics to potential therapies

Imarisio, S., Carmichael, J., Korolchuk, V., Chen, C.-W., Saiki, S., Rose, C., Krishna, G., Davies, J. E., Ttofi, E., Underwood, B. R., et al. (2008) · Biochemical Journal · doi:10.1042/bj20071619 · Cited by 409 · see citing works

Mitophagy in neurodegeneration and aging

Fivenson, E. M., Lautrup, S., Sun, N., Scheibye-Knudsen, M., Stevnsner, T., Nilsen, H., Bohr, V. A., & Fang, E. F. (2017) · Neurochemistry International · doi:10.1016/j.neuint.2017.02.007 · Cited by 364 · see citing works

Transcriptional coregulators in the control of energy homeostasis

Feige, J. N., & Auwerx, J. (2007) · Trends in Cell Biology · doi:10.1016/j.tcb.2007.04.001 · Cited by 325 · see citing works

Mitochondrial Approaches for Neuroprotection

Chaturvedi, R. K., & Beal, M. F. (2008) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1427.027 · Cited by 294 · see citing works

Antioxidant and bioenergetic coupling between neurons and astrocytes

Fernandez-Fernandez, S., Almeida, A., & Bolaños, J. P. (2012) · Biochemical Journal · doi:10.1042/bj20111943 · Cited by 247 · see citing works

Mitochondrial Medicine for Aging and Neurodegenerative Diseases

Reddy, P. H. (2008) · NeuroMolecular Medicine · doi:10.1007/s12017-008-8044-z · Cited by 230 · see citing works

The coordination of nuclear and mitochondrial communication during aging and calorie restriction

Finley, L. W. S., & Haigis, M. C. (2009) · Ageing Research Reviews · doi:10.1016/j.arr.2009.03.003 · Cited by 207 · see citing works

A single nucleotide polymorphism in the coding region of PGC-1α is a male-specific modifier of Huntington disease age-at-onset in a large European cohort

For the European Huntington Disease Network, Weydt, P., Soyal, S. M., Landwehrmeyer, G. B., & Patsch, W. (2014) · BMC Neurology · doi:10.1186/1471-2377-14-1 · Cited by 206 · see citing works

Hypothalamic Alterations in Neurodegenerative Diseases and Their Relation to Abnormal Energy Metabolism

Vercruysse, P., Vieau, D., Blum, D., Petersén, Å., & Dupuis, L. (2018) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2018.00002 · Cited by 172 · see citing works

Mitochondria and Huntington's Disease Pathogenesis: Insight from Genetic and Chemical Models

Browne, S. E. (2008) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1427.018 · Cited by 176 · see citing works

Huntington's disease: The current state of research with peripheral tissues

Sassone, J., Colciago, C., Cislaghi, G., Silani, V., & Ciammola, A. (2009) · Experimental Neurology · doi:10.1016/j.expneurol.2009.05.012 · Cited by 175 · see citing works

Adult Type 3 Adenylyl Cyclase–Deficient Mice Are Obese

Wang, Z., Li, V., Chan, G. C. K., Phan, T., Nudelman, A. S., Xia, Z., & Storm, D. R. (2009) · PLoS ONE · doi:10.1371/journal.pone.0006979 · Cited by 159 · see citing works

Turn up the power - pharmacological activation of mitochondrial biogenesis in mouse models

Komen, J. C., & Thorburn, D. R. (2014) · British Journal of Pharmacology · doi:10.1111/bph.12413 · Cited by 149 · see citing works

PGC-1α, a New Therapeutic Target in Huntington's Disease?

McGill, J. K., & Beal, M. F. (2006) · Cell · doi:10.1016/j.cell.2006.10.023 · Cited by 131 · see citing works

Nicotinamide improves motor deficits and upregulates PGC-1α and BDNF gene expression in a mouse model of Huntington's disease

Hathorn, T., Snyder-Keller, A., & Messer, A. (2011) · Neurobiology of Disease · doi:10.1016/j.nbd.2010.08.017 · Cited by 123 · see citing works

Genetic Determinants of Human Health Span and Life Span: Progress and New Opportunities

Martin, G. M., Bergman, A., & Barzilai, N. (2007) · PLoS Genetics · doi:10.1371/journal.pgen.0030125 · Cited by 120 · see citing works

IRS2 increases mitochondrial dysfunction and oxidative stress in a mouse model of Huntington disease

Sadagurski, M., Cheng, Z., Rozzo, A., Palazzolo, I., Kelley, G. R., Dong, X., Krainc, D., & White, M. F. (2011) · Journal of Clinical Investigation · doi:10.1172/jci46305 · Cited by 107 · see citing works

Genome-wide association study in essential tremor identifies three new loci

Müller, S. H., Girard, S. L., Hopfner, F., Merner, N. D., Bourassa, C. V., Lorenz, D., Clark, L. N., Tittmann, L., Soto-Ortolaza, A. I., Klebe, S., et al. (2016) · Brain · doi:10.1093/brain/aww242 · Cited by 88 · see citing works

Treating neurodegeneration by modifying mitochondria: potential solutions to a “complex” problem

Swerdlow, R. H. (2007) · Antioxidants & Redox Signaling · doi:10.1089/ars.2007.1676 · Cited by 81 · see citing works

The influence of synaptic activity on neuronal health

Bell, K. F., & Hardingham, G. E. (2011) · Current Opinion in Neurobiology · doi:10.1016/j.conb.2011.01.002 · Cited by 77 · see citing works

PGC-1α, Sirtuins and PARPs in Huntington’s Disease and Other Neurodegenerative Conditions: NAD+ to Rule Them All

Lloret, A., & Beal, M. F. (2019) · Neurochemical Research · doi:10.1007/s11064-019-02809-1 · Cited by 52 · see citing works

PGC-1α signaling coordinates susceptibility to metabolic and oxidative injury in the inner retina

Guo, X., Dason, E. S., Zanon-Moreno, V., Jiang, Q., Nahirnyj, A., Chan, D., Flanagan, J. G., & Sivak, J. M. (2014) · The American Journal of Pathology · doi:10.1016/j.ajpath.2013.12.012 · Cited by 47 · see citing works

Comparison of sirtuin 3 levels in ALS and Huntington's disease-differential effects in human tissue samples vs. transgenic mouse models

Buck, E., Bayer, H., Lindenberg, K. S., Hanselmann, J., Pasquarelli, N., Ludolph, A. C., Weydt, P., & Witting, A. (2017) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2017.00156 · Cited by 36 · see citing works

Generation of a neuro-specific microarray reveals novel differentially expressed noncoding RNAs in mouse models for neurodegenerative diseases

Gstir, R., Schafferer, S., Scheideler, M., Misslinger, M., Griehl, M., Daschil, N., Humpel, C., Obermair, G. J., Schmuckermair, C., Striessnig, J., et al. (2014) · RNA · doi:10.1261/rna.047225.114 · Cited by 36 · see citing works

Huntingtin Acts in the Nucleus to Induce Apoptosis but Death Does Not Correlate with the Formation of Intranuclear Inclusions

Saudou, F., Finkbeiner, S. M., Devys, D., & Greenberg, M. E. (1998) · Cell · doi:10.1016/s0092-8674(00)81782-1 · Cited by 1,598 · see citing works

Analysis of the huntingtin (HTT)-dependent transcriptome in brain from the R6/1 mouse model of Huntington’s disease

Jones, L. (2019) · Signaling Pathways Project Datasets · doi:10.1621/rklfkknune

Assessments of potential interactions of huntingtin-associated-protein-1 in the R6/2 mouse model of Huntington's disease

Stenske, M., & Dunbar, G. L. (2004)

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Hersch, S. M., Gutekunst, C., & Ferrante, R. J. (1997) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-199705000-00081

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Yan, S., Tu, Z., Liu, Z., Fan, N., Yang, H., Yang, S., Yang, W., Zhao, Y., Ouyang, Z., Lai, C., et al. (2018) · Cell · doi:10.1016/j.cell.2018.03.005 · Cited by 339 · see citing works