The Energetics of Huntington's Disease

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

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

Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain

DiFiglia, M., Sapp, E., Chase, K. O., Davies, S. W., Bates, G. P., Vonsattel, J., & D, N. A. M. (1997) · Science · doi:10.1126/science.277.5334.1990 · Cited by 2,849 · see citing works

Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD Mutation

Davies, S. W., Turmaine, M., Cozens, B. A., DiFiglia, M., Sharp, A. H., Ross, C. A., Scherzinger, E., Wanker, E. E., Mangiarini, L., & Bates, G. P. (1997) · Cell · doi:10.1016/s0092-8674(00)80513-9 · Cited by 2,188 · 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

Loss of Huntingtin-Mediated BDNF Gene Transcription in Huntington's Disease

Zuccato, C., Ciammola, A., Rigamonti, D., Leavitt, B. R., Goffredo, D., Conti, L., MacDonald, M. E., Friedlander, R. M., Silani, V., Hayden, M. R., et al. (2001) · Science · doi:10.1126/science.1059581 · Cited by 1,319 · see citing works

Huntington Disease

Vonsattel, J. P., & DiFiglia, M. (1998) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-199805000-00001 · Cited by 1,305 · see citing works

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

Steffan, J. S., Bodai, L., Pallos, J., Poelman, M., McCampbell, A., Apostol, B. L., Kazantsev, A., Schmidt, E., Zhu, Y.-Z., Greenwald, M., et al. (2001) · Nature · doi:10.1038/35099568 · Cited by 1,228 · see citing works

Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy

Ravikumar, B. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.9.1107 · Cited by 1,099 · see citing works

Reversal of Neuropathology and Motor Dysfunction in a Conditional Model of Huntington's Disease

Yamamoto, A., Lucas, J. J., & Hen, R. (2000) · Cell · doi:10.1016/s0092-8674(00)80623-6 · Cited by 1,068 · see citing works

Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines

Panov, A. V., Gutekunst, C.-A., Leavitt, B. R., Hayden, M. R., Burke, J. R., Strittmatter, W. J., & Greenamyre, J. T. (2002) · Nature Neuroscience · doi:10.1038/nn884 · Cited by 1,030 · see citing works

Glutamate becomes neurotoxic via the N-methyl-d-aspartate receptor when intracellular energy levels are reduced

Novelli, A., Reilly, J. A., Lysko, P. G., & Henneberry, R. C. (1988) · Brain Research · doi:10.1016/0006-8993(88)90765-2 · Cited by 961 · 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

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

Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons

DiFiglia, M., Sapp, E., Chase, K., Schwarz, C., Meloni, A., Young, C., Martin, E., Vonsattel, J.-P., Carraway, R., Reeves, S. A., et al. (1995) · Neuron · doi:10.1016/0896-6273(95)90346-1 · Cited by 749 · see citing works

Inactivation of the Mouse Huntington's Disease Gene Homolog <i>Hdh</i>

Duyao, M. P., Auerbach, A. B., Ryan, A., Persichetti, F., Barnes, G. T., McNeil, S. M., Ge, P., Vonsattel, J.-P., Gusella, J. F., Joyner, A. L., et al. (1995) · Science · doi:10.1126/science.7618107 · Cited by 723 · 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

Sp1 and TAFII130 Transcriptional Activity Disrupted in Early Huntington's Disease

Dunah, A. W., Jeong, H., Griffin, A. M., Kim, Y., Standaert, D. G., Hersch, S. M., Mouradian, M. M., Young, A. B., Tanese, N., & Krainc, D. (2002) · Science · doi:10.1126/science.1072613 · Cited by 708 · see citing works

Increased Sensitivity to N-Methyl-D-Aspartate Receptor-Mediated Excitotoxicity in a Mouse Model of Huntington's Disease

Zeron, M. M., Hansson, O., Chen, N., Wellington, C. L., Leavitt, B. R., Brundin, P., Hayden, M. R., & Raymond, L. A. (2002) · Neuron · doi:10.1016/s0896-6273(02)00615-3 · Cited by 600 · see citing works

Neurological abnormalities in a knock-in mouse model of Huntington's disease

Lin, C. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.2.137 · Cited by 592 · see citing works

Alternative excitotoxic hypotheses

Albin, R. L., & Greenamyre, J. T. (1992) · Neurology · doi:10.1212/wnl.42.4.733 · Cited by 574 · cited 1× · see citing works

Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion

White, J. K., Auerbach, W., Duyao, M. P., Vonsattel, J., Gusella, J. F., Joyner, A. L., & MacDonald, M. E. (1997) · Nature Genetics · doi:10.1038/ng1297-404 · Cited by 536 · see citing works

Neuroprotective Effects of Creatine in a Transgenic Mouse Model of Huntington's Disease

Ferrante, R. J., Andreassen, O. A., Jenkins, B. G., Dedeoglu, A., Kuemmerle, S., Kubilus, J. K., Kaddurah-Daouk, R., Hersch, S. M., & Beal, M. F. (2000) · The Journal of Neuroscience · doi:10.1523/jneurosci.20-12-04389.2000 · Cited by 522 · see citing works

Widespread expression of Huntington's disease gene (IT15) protein product

Sharp, A. H., Loev, S. J., Schilling, G., Li, S.-H., Li, X.-J., Bao, J., Wagster, M. V., Kotzuk, J. A., Steiner, J. P., Lo, A., et al. (1995) · Neuron · doi:10.1016/0896-6273(95)90345-3 · Cited by 513 · see citing works

Oxidative stress in Huntington’s disease

Browne, S. E., Ferrante, R. J., & Beal, M. F. (1999) · Brain Pathology · doi:10.1111/j.1750-3639.1999.tb00216.x · Cited by 508 · see citing works

Isolated Cerebral and Cerebellar Mitochondria Produce Free Radicals when Exposed to Elevated Ca2+ and Na+: Implications for Neurodegeneration

Dykens, J. A. (1994) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.1994.63020584.x · Cited by 493 · see citing works

Effect of Peroxynitrite on the Mitochondrial Respiratory Chain: Differential Susceptibility of Neurones and Astrocytes in Primary Culture

Bolaños, J. P., Heales, S., Land, J. M., & Clark, J. B. (1995) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.1995.64051965.x · Cited by 492 · 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

3-Nitropropionic Acid - Exogenous Animal Neurotoxin and Possible Human Striatal Toxin

Ludolph, A. C., He, F., Spencer, P. S., Hammerstad, J. P., & Sabri, M. I. (1991) · Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques · doi:10.1017/s0317167100032212 · Cited by 484 · see citing works

Evidence for irnnairment of energy metabofism in vivo in Huntington's disease using localized 1H NMR spectroscopy

Jenkins, B. G., Koroshetz, W. J., Beal, M. F., & Rosen, B. R. (1993) · Neurology · doi:10.1212/wnl.43.12.2689 · Cited by 479 · see citing works

Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice

Wheeler, V. C. (2000) · Human Molecular Genetics · doi:10.1093/hmg/9.4.503 · Cited by 471 · see citing works

Neuroprotective Effects of Creatine and Cyclocreatine in Animal Models of Huntington’s Disease

Matthews, R. T., Yang, L., Jenkins, B. G., Ferrante, R. J., Rosen, B. R., Kaddurah-Daouk, R. F., & Beal, M. F. (1998) · Journal of Neuroscience · doi:10.1523/jneurosci.18-01-00156.1998 · Cited by 431 · see citing works

Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH

Burke, J. R., Enghild, J. J., Martin, M. E., Jou, Y., Myers, R., Roses, A. D., Vance, J. M., & Strittmatter, W. J. (1996) · Nature Medicine · doi:10.1038/nm0396-347 · Cited by 430 · see citing works

Dietary restriction normalizes glucose metabolism and BDNF levels, slows disease progression, and increases survival in huntingtin mutant mice

Duan, W., Guo, Z., Jiang, H., Ware, M., Li, X.-J., & Mattson, M. P. (2003) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0536856100 · Cited by 428 · see citing works

Proteases Acting on Mutant Huntingtin Generate Cleaved Products that Differentially Build Up Cytoplasmic and Nuclear Inclusions

Lunkes, A., Lindenberg, K. S., Ben-Haı̈em, L., Weber, C., Devys, D., Landwehrmeyer, G. B., Mandel, J., & Trottier, Y. (2002) · Molecular Cell · doi:10.1016/s1097-2765(02)00602-0 · Cited by 413 · see citing works

Wild-Type Huntingtin Protects from Apoptosis Upstream of Caspase-3

Rigamonti, D., Bauer, J. H., De‐Fraja, C., Conti, L., Sipione, S., Sciorati, C., Clementi, E., Hackam, A. S., Hayden, M. R., Li, Y., et al. (2000) · Journal of Neuroscience · doi:10.1523/jneurosci.20-10-03705.2000 · Cited by 406 · see citing works

Energy metabolism defects in Huntington's disease and effects of coenzyme Q₁₀

Koroshetz, W. J., Jenkins, B. G., Rosen, B. R., & Beal, M. F. (1997) · Annals of Neurology · doi:10.1002/ana.410410206 · Cited by 405 · see citing works

Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA

Reddy, P. H., Williams, M., Charles, V., Garrett, L. J., Pike-Buchanan, L., Whetsell, W. O., Miller, G., & Tagle, D. A. (1998) · Nature Genetics · doi:10.1038/2510 · Cited by 399 · see citing works

NT-3 and BDNF protect CNS neurons against metabolic/excitotoxic insults

Cheng, B., & Mattson, M. P. (1994) · Brain Research · doi:10.1016/0006-8993(94)91857-0 · Cited by 394 · see citing works

Therapeutic Effects of Coenzyme Q₁₀and Remacemide in Transgenic Mouse Models of Huntington's Disease

Ferrante, R. J., Andreassen, O. A., Dedeoglu, A., Ferrante, K. L., Jenkins, B. G., Hersch, S. M., & Beal, M. F. (2002) · The Journal of Neuroscience · doi:10.1523/jneurosci.22-05-01592.2002 · Cited by 391 · see citing works

Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain

Luthi‐Carter, R. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.17.1911 · Cited by 364 · see citing works

Transgenic rat model of Huntington's disease

von Hörsten, S., Schmitt, I., Nguyen, H. P., Holzmann, C., Schmidt, T., Walther, T., Bäder, M., Pabst, R., Kobbe, P., Krotova, J., et al. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg075 · Cited by 361 · see citing works

Transient and Progressive Electrophysiological Alterations in the Corticostriatal Pathway in a Mouse Model of Huntington's Disease

Cepeda, C., Hurst, R. S., Calvert, C. R., Hernández, E., Nguyen, O. K., Jocoy, E. L., Christian, L. J., Ariano, M. A., & Levine, M. S. (2003) · Journal of Neuroscience · doi:10.1523/jneurosci.23-03-00961.2003 · Cited by 356 · see citing works

Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington's disease

Levine, M. S., Klapstein, G. J., Koppel, A. B., Gruen, E., Cepeda, C., Vargas, M. E., Jokel, E. S., Carpenter, E. M., Zanjani, H., Hurst, R. S., et al. (1999) · Journal of Neuroscience Research · doi:10.1002/(sici)1097-4547(19991115)58:4 · Cited by 351 · see citing works

Huntingtin localization in brains of normal and Huntington's disease patients

Sapp, E., Schwarz, C., Chase, K. O., Bhide, P. G., Young, A. B., Penney, J. B., Vonsattel, J. P., D, N. A. M., & DiFiglia, M. (1997) · Annals of Neurology · doi:10.1002/ana.410420411 · Cited by 350 · see citing works

Brain-derived neurotrophic factor in Huntington disease

Ferrer, I., Goutan, E., Marı́n, C., Rey, M. J., & Ribalta, T. (2000) · Brain Research · doi:10.1016/s0006-8993(00)02237-x · Cited by 333 · see citing works

Involvement of Free Radicals in Excitotoxicity In Vivo

Schulz, J. B., Henshaw, D. R., Siwek, D. F., Jenkins, B. G., Ferrante, R. J., Cipolloni, P. B., Kowall, N. W., Rosen, B. R., & Beal, M. F. (1995) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.1995.64052239.x · Cited by 322 · see citing works

Striatal glucose metabolism and dopamine D2 receptor binding in asymptomatic gene carriers and patients with Huntington's disease

Antonini, A., Leenders, K. L., Spiegel, R., Meier, D., Vontobel, P., Weigell-Weber, M., Sánchez‐Pernaute, R., de Yébenez, J. G., Boesiger, P., Weindl, A., et al. (1996) · Brain · doi:10.1093/brain/119.6.2085 · Cited by 317 · see citing works

A randomized, placebo-controlled trial of coenzyme Q ₁₀ and remacemide in Huntington’s disease

The Huntington Study Group (2001) · Neurology · doi:10.1212/wnl.57.3.397 · Cited by 312 · see citing works

Polyglutamine-mediated dysfunction and apoptotic death of a Caenorhabditis elegans sensory neuron

Faber, P. W., Alter, J. R., MacDonald, M. E., & Hart, A. C. (1999) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.96.1.179 · Cited by 306 · see citing works

Mutant Huntingtin Expression in Clonal Striatal Cells: Dissociation of Inclusion Formation and Neuronal Survival by Caspase Inhibition

Kim, M., Lee, H.-S., Laforet, G. A., McIntyre, C., Martin, E. J., Chang, P., Kim, T. W., Williams, M. J., Reddy, P. H., Tagle, D., et al. (1999) · Journal of Neuroscience · doi:10.1523/jneurosci.19-03-00964.1999 · Cited by 305 · see citing works

Creatine Increases Survival and Delays Motor Symptoms in a Transgenic Animal Model of Huntington's Disease

Andreassen, O. A., Dedeoglu, A., Ferrante, R. J., Jenkins, B. G., Ferrante, K. L., Thomas, M., Friedlich, A., Browne, S. E., Schilling, G., Borchelt, D. R., et al. (2001) · Neurobiology of Disease · doi:10.1006/nbdi.2001.0406 · Cited by 293 · see citing works

Striatal and nigral neuron subpopulations in rigid Huntington's disease: Implications for the functional anatomy of chorea and rigidity‐akinesia

Albin, R. L., Reiner, A. J., Anderson, K. D., Penney, J. B., & Young, A. B. (1990) · Annals of Neurology · doi:10.1002/ana.410270403 · Cited by 292 · see citing works

Changes in Cortical and Striatal Neurons Predict Behavioral and Electrophysiological Abnormalities in a Transgenic Murine Model of Huntington's Disease

Laforet, G. A., Sapp, E., Chase, K. O., McIntyre, C., Boyce, F. M., Campbell, M. L., Cadigan, B. A., Warzecki, L., Tagle, D. A., Reddy, P. H., et al. (2001) · Journal of Neuroscience · doi:10.1523/jneurosci.21-23-09112.2001 · Cited by 291 · see citing works

Weight loss in early stage of Huntington’s disease

Djoussé, L., Knowlton, B. A., Cupples, L. A., Marder, K. S., Shoulson, I., & Myers, R. H. (2002) · Neurology · doi:10.1212/01.wnl.0000031791.10922.cf · Cited by 284 · see citing works

Lessons from animal models of Huntington's disease

Rubinsztein, D. C. (2002) · Trends in Genetics · doi:10.1016/s0168-9525(01)02625-7 · Cited by 281 · see citing works

Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice

Ginés, S. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg046 · Cited by 279 · see citing works

CORTICAL AND SUBCORTICAL GLUCOSE CONSUMPTION MEASURED BY PET IN PATIENTS WITH HUNTINGTON'S DISEASE

Kuwert, T., Lange, H. W., Langen, K., Herzog, H. R., Aulich, A., & Feinendegen, L. E. (1990) · Brain · doi:10.1093/brain/113.5.1405 · Cited by 274 · see citing works

Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease

Wyttenbach, A. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.17.1829 · Cited by 274 · see citing works

Mitochondrial involvement in brain function and dysfunction: relevance to aging, neurodegenerative disorders and longevity

Calabrese, V., Scapagnini, G., Giuffrida Stella, A. M., Bates, T. E., & Clark, J. B. (2001) · Neurochemical Research · doi:10.1023/a:1010955807739 · Cited by 263 · see citing works

A Huntington's Disease CAG Expansion at the Murine Hdh Locus Is Unstable and Associated with Behavioural Abnormalities in Mice

Shelbourne, P., Killeen, N. P., Hevner, R. F., Johnston, H. M., Tecott, L. H., Lewandoski, M. B., Ennis, M., Ramirez, L., Li, Z., Iannicola, C., et al. (1999) · Human Molecular Genetics · doi:10.1093/hmg/8.5.763 · Cited by 263 · see citing works

Mutant Huntingtin Causes Context-Dependent Neurodegeneration in Mice with Huntington's Disease

Yu, Z.-X., Li, S.-H., Evans, J., Pillarisetti, A., He, L., & Li, X. (2003) · Journal of Neuroscience · doi:10.1523/jneurosci.23-06-02193.2003 · Cited by 260 · see citing works

When more is less: Pathogenesis of glutamine repeat neurodegenerative diseases

Ross, C. A. (1995) · Neuron · doi:10.1016/0896-6273(95)90138-8 · Cited by 260 · see citing works

Increased oxidative damage to DNA in a transgenic mouse model of Huntington's disease

Bogdanov, M., Andreassen, O. A., Dedeoglu, A., Ferrante, R. J., & Beal, M. F. (2001) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2001.00689.x · Cited by 248 · see citing works

Abnormalities of Striatal Projection Neurons andN-Methyl-D-Aspartate Receptors in Presymptomatic Huntington's Disease

Albin, R. L., Young, A. B., Penney, J. B., et al. (1990) · New England Journal of Medicine · doi:10.1056/nejm199005033221807 · Cited by 245 · see citing works

Excitotoxin Lesions in Primates as a Model for Huntington's Disease: Histopathologic and Neurochemical Characterization

Ferrante, R. J., Kowall, N. W., Cipolloni, P. B., Storey, E., & Beal, M. F. (1993) · Experimental Neurology · doi:10.1006/exnr.1993.1006 · Cited by 244 · see citing works

Potential for creatine and other therapies targeting cellular energy dysfunction in neurological disorders

Tarnopolsky, M. A., & Beal, M. F. (2001) · Annals of Neurology · doi:10.1002/ana.1028 · Cited by 244 · see citing works

1 H NMR spectroscopy studies of Huntington's disease

Jenkins, B. G., Rosas, H. D., Chen, Y. I., Makabe, T., Myers, R. H., MacDonald, M. E., Rosen, B. R., Beal, M. F., & Koroshetz, W. J. (1998) · Neurology · doi:10.1212/wnl.50.5.1357 · Cited by 241 · see citing works

Evidence for a defect in NADH

Parker, W. D., Boyson, S. J., LUDER, A. S., & Parks, J. K. (1990) · Neurology · doi:10.1212/wnl.40.8.1231 · Cited by 230 · see citing works

Early transcriptional profiles in huntingtin-inducible striatal cells by microarray analyses

Sipione, S., Rigamonti, D., Valenza, M., Zuccato, C., Conti, L., Pritchard, J., Kooperberg, C. L., Olson, J. M., & Cattaneo, E. F. (2014) · Human Molecular Genetics · doi:10.1093/hmg/ddu539 · Cited by 222 · see citing works

Oxidative damage to mitochondrial DNA in Huntington's disease parietal cortex

Polidori, M. C., Mecocci, P., Browne, S. E., Senin, U., & Beal, M. F. (1999) · Neuroscience Letters · doi:10.1016/s0304-3940(99)00578-9 · Cited by 211 · see citing works

Polyglutamine and transcription: gene expression changes shared by DRPLA and Huntington's disease mouse models reveal context-independent effects

Luthi‐Carter, R. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.17.1927 · Cited by 211 · see citing works

Health implications of creatine: can oral creatine supplementation protect against neurological and atherosclerotic disease?

Wyss, M., & Schulze, A. (2002) · Neuroscience · doi:10.1016/s0306-4522(02)00088-x · Cited by 210 · see citing works

Partial Inhibition of Brain Succinate Dehydrogenase by 3‐Nitropropionic Acid Is Sufficient to Initiate Striatal Degeneration in Rat

Brouillet, E., Guyot, M., Mittoux, V., Altairac, S., Condé, F., Palfi, S., & Hantraye, P. (1998) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.1998.70020794.x · Cited by 210 · see citing works

Early transcriptional profiles in huntingtin-inducible striatal cells by microarray analyses

Sipione, S. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.17.1953 · Cited by 209 · see citing works

Huntington's disease progression

Andrews, T., Weeks, R. A., Turjanski, N., Gunn, R. N., Watkins, L., Sahakian, B. J., Hodges, J. R., Rosser, A., Wood, N. W., & Brooks, D. J. (1999) · Brain · doi:10.1093/brain/122.12.2353 · Cited by 205 · see citing works

Apoptosis in Huntington's disease

Hickey, M. A., & Chesselet, M.-F. (2003) · Progress in Neuro-Psychopharmacology and Biological Psychiatry · doi:10.1016/s0278-5846(03)00021-6 · Cited by 197 · see citing works

Early Degenerative Changes in Transgenic Mice Expressing Mutant Huntingtin Involve Dendritic Abnormalities but No Impairment of Mitochondrial Energy Production

Guidetti, P., Charles, V., Chen, E., Reddy, P. H., Kordower, J. H., Whetsell, W. O., Schwarcz, R. M., & Tagle, D. A. (2001) · Experimental Neurology · doi:10.1006/exnr.2000.7626 · Cited by 197 · see citing works

Cellular Localization of Huntingtin in Striatal and Cortical Neurons in Rats: Lack of Correlation with Neuronal Vulnerability in Huntington’s Disease

Fusco, F. R., Chen, Q., L’Amoreaux, W. J., Figueredo-Cardenas, G., Jiao, Y., Coffman, J. A., Surmeier, D. J., Honig, M. G., Carlock, L. R., & Reiner, A. J. (1999) · Journal of Neuroscience · doi:10.1523/jneurosci.19-04-01189.1999 · Cited by 192 · see citing works

Nuclear Targeting of Mutant Huntingtin Increases Toxicity

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