Oxidative stress in Huntington’s disease

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

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The functional anatomy of basal ganglia disorders

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Functional architecture of basal ganglia circuits: neural substrates of parallel processing

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

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

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Protein Oxidation and Aging

Stadtman, E. R. (1992) · Science · doi:10.1126/science.1355616 · Cited by 2,701 · see citing works

Neuropathological Classification of Huntingtonʼs Disease

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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

Calcium-mediated neurotoxicity: relationship to specific channel types and role in ischemic damage

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

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Peroxynitrite-mediated tyrosine nitration catalyzed by superoxide dismutase

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The Basal Ganglia and Adaptive Motor Control

Graybiel, A. M., Aosaki, T., Flaherty, A. W., & Kimura, M. (1994) · Science · doi:10.1126/science.8091209 · Cited by 1,324 · see citing works

Huntington Disease

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Huntingtin-Encoded Polyglutamine Expansions Form Amyloid-like Protein Aggregates In Vitro and In Vivo

Scherzinger, E., Lurz, R., Turmaine, M., Mangiarini, L., Hollenbach, B., Hasenbank, R., Bates, G. P., Davies, S. W., Lehrach, H., & Wanker, E. E. (1997) · Cell · doi:10.1016/s0092-8674(00)80514-0 · Cited by 1,291 · cited 1× · see citing works

Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid

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MITOCHONDRIAL DNA MUTATIONS AS AN IMPORTANT CONTRIBUTOR TO AGEING AND DEGENERATIVE DISEASES

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The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease

Andrew, S. E., Goldberg, Y. P., Kremer, B. P. H., Telenius, H., Theilmann, J., Adam, S., Starr, E. M., Squitieri, F., Lin, B., Kalchman, M. A., et al. (1993) · Nature Genetics · doi:10.1038/ng0893-398 · Cited by 1,135 · see citing works

Nitric Oxide Activation of Poly(ADP-Ribose) Synthetase in Neurotoxicity

Zhang, J., Dawson, V. L., Dawson, T. M., & Snyder, S. H. (1994) · Science · doi:10.1126/science.8080500 · Cited by 1,118 · see citing works

Oxidative damage to DNA during aging: 8-hydroxy-2'-deoxyguanosine in rat organ DNA and urine.

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Trinucleotide repeat length instability and age of onset in Huntington's disease

Duyao, M., Ambrose, C., Myers, R., Novelletto, A., Persichetti, F., Frontali, M., Folstein, S., Ross, C., Franz, M., Abbott, M., et al. (1993) · Nature Genetics · doi:10.1038/ng0893-387 · Cited by 1,089 · see citing works

Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases.

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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

Differential loss of striatal projection neurons in Huntington disease.

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

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Does impairment of energy metabolism result in excitotoxic neuronal death in neurodegenerative illnesses?

Beal, M. F. (1992) · Annals of Neurology · doi:10.1002/ana.410310202 · Cited by 956 · 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

Intranuclear Inclusions of Expanded Polyglutamine Protein in Spinocerebellar Ataxia Type 3

Paulson, H. L., Perez, M. K., Trottier, Y., Trojanowski, J. Q., Subramony, S. H., Das, S. S., Vig, P. J. S., Mandel, J., Fischbeck, K. H., & Pittman, R. N. (1997) · Neuron · doi:10.1016/s0896-6273(00)80943-5 · Cited by 830 · see citing works

Mitochondrial production of reactive oxygen species in cortical neurons following exposure to N-methyl-D-aspartate

Dugan, L. L., Sensi, S., Canzoniero, L. M. T., Handran, S. D., Rothman, S., Lin, T., Goldberg, M. P., & Choi, D. W. (1995) · Journal of Neuroscience · doi:10.1523/jneurosci.15-10-06377.1995 · Cited by 762 · 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

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

Evidence for Degenerative and Regenerative Changes in Neostriatal Spiny Neurons in Huntington's Disease

Graveland, G. A., Williams, R. S., & DiFiglia, M. (1985) · Science · doi:10.1126/science.3155875 · Cited by 612 · see citing works

Evidence for apoptotic cell death in Huntington disease and excitotoxic animal models

Portera‐Cailliau, C., Hedreen, J. C., Price, D., & Koliatsos, V. (1995) · Journal of Neuroscience · doi:10.1523/jneurosci.15-05-03775.1995 · Cited by 607 · 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

Alternative excitotoxic hypotheses

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

Do defecs in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases?

Beal, M. F., Hyman, B. T., & Koroshetz, W. J. (1993) · Trends in Neurosciences · doi:10.1016/0166-2236(93)90117-5 · Cited by 574 · 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

Polyglutamine-Expanded Human Huntingtin Transgenes Induce Degeneration of Drosophila Photoreceptor Neurons

Jackson, G. R., Salecker, I., Dong, X., Yao, X., Arnheim, N., Faber, P. W., MacDonald, M. E., & Zipursky, S. L. (1998) · Neuron · doi:10.1016/s0896-6273(00)80573-5 · Cited by 535 · see citing works

Calcium transport and buffering in neurons

Blaustein, M. P. (1988) · Trends in Neurosciences · doi:10.1016/0166-2236(88)90195-6 · Cited by 516 · 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

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

Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form

Trottier, Y., Devys, D., Imbert, G., Saudou, F., Gourfinkel‐An, I., Lutz, Y., Weber, C., Agid, Y., Hirsch, É. C., & Mandel, J. (1995) · Nature Genetics · doi:10.1038/ng0595-104 · Cited by 452 · see citing works

Morphometric Demonstration of Atrophic Changes in the Cerebral Cortex, White Matter, and Neostriatum in Huntington's Disease

de la Monte, S. M., Vonsattel, J., & Richardson, E. P. (1988) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-198809000-00003 · Cited by 434 · see citing works

Metabotropic glutamate receptors: a new target for the therapy of neurodegenerative disorders?

Nicoletti, F., Bruno, V., Copani, A., Casabona, G., & Knöpfel, T. (1996) · Trends in Neurosciences · doi:10.1016/s0166-2236(96)20019-0 · Cited by 433 · 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

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

NMDA Receptor Losses in Putamen from Patients with Huntington's Disease

Young, A. B., Greenamyre, J. T., Hollingsworth, Z. R., Albin, R. L., D’Amato, C. J., Shoulson, I., & Penney, J. B. (1988) · Science · doi:10.1126/science.2841762 · Cited by 394 · see citing works

Lipofuscin: Mechanisms of formation and increase with age

TERMAN, A., & BRUNK, U. T. (1998) · APMIS · doi:10.1111/j.1699-0463.1998.tb01346.x · Cited by 387 · see citing works

Age-dependent vulnerability of the striatum to the mitochondrial toxin 3-nitropropionic acid

Brouillet, E., Jenkins, B. G., Hyman, B. T., Ferrante, R. J., Kowall, N. W., Srivastava, R., Roy, D. S., Rosen, B. R., & Beal, M. F. (1993) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1993.tb05859.x · Cited by 367 · see citing works

Reduced Cerebral Glucose Metabolism in Asymptomatic Subjects at Risk for Huntington's Disease

Mazziotta, J. C., Phelps, M. E., Pahl, J. J., Huang, S., Baxter, L. R., Riege, W. H., Hoffman, J. M., Kuhl, D. E., Lanto, A. B., Wapenski, J. A., et al. (1987) · New England Journal of Medicine · doi:10.1056/nejm198702123160701 · Cited by 314 · see citing works

Identification and localization of huntingtin in brain and human lymphoblastoid cell lines with anti-fusion protein antibodies.

Gutekunst, C., Levey, A. I., Heilman, C. J., Whaley, W. L., Yi, H. L., Nash, N. R., Rees, H. D., Madden, J. J., & Hersch, S. M. (1995) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.92.19.8710 · Cited by 305 · see citing works

Morphologic and Histochemical Characteristics of a Spared Subset of Striatal Neurons in Huntington's Disease

Ferrante, R. J., Kowall, N. W., Beal, M. F., Martin, J. B., Bird, E. D., & Richardson, E. P. (1987) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-198701000-00002 · Cited by 294 · 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

Neurons Containing NADPH-Diaphorase Are Selectively Resistant to Quinolinate Toxicity

Koh, J., Peters, S., & Choi, D. W. (1986) · Science · doi:10.1126/science.2875522 · Cited by 288 · 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

Manganese Superoxide Dismutase Protects nNOS Neurons from NMDA and Nitric Oxide-Mediated Neurotoxicity

Gonzalez‐Zulueta, M., Ensz, L. M., Mukhina, G. L., Lebovitz, R. M., Zwacka, R. M., Engelhardt, J. F., Oberley, L. W., Dawson, V. L., & Dawson, T. M. (1998) · Journal of Neuroscience · doi:10.1523/jneurosci.18-06-02040.1998 · Cited by 265 · see citing works

Regional Mitochondrial Respiratory Activity in Huntington's Disease Brain

Brennan, W. A., Bird, E. D., & Aprille, J. R. (1985) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1985.tb07192.x · Cited by 265 · 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

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

Decreased Neuronal and Increased Oligodendroglial Densities in Huntington's Disease Caudate Nucleus

Myers, R. H., Vonsattel, J. P., Paskevich, P. A., Kiely, D. K., Stevens, T. J., Cupples, L. A., Richardson, E. P., & Bird, E. D. (1991) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-199111000-00005 · Cited by 239 · see citing works

Huntington's disease gene: Regional and cellular expression in brain of normal and affected individuals

Landwehrmeyer, G. B., McNeil, S. M., Dure, L. S., Ge, P., Aizawa, H., Huang, Q. M., Ambrose, C. M., Duyao, M. P., Bird, E. D., Bonilla, E., et al. (1995) · Annals of Neurology · doi:10.1002/ana.410370213 · Cited by 222 · see citing works

Selective sparing of NADPH‐diaphorase–somatostatin–neuropeptide Y neurons in ischemic gerbil striatum

Uemura, Y., Kowall, N. W., & Beal, M. F. (1990) · Annals of Neurology · doi:10.1002/ana.410270606 · Cited by 217 · see citing works

Inhibition of Succinate Dehydrogenase by Malonic Acid Produces an “Excitotoxic” Lesion in Rat Striatum

Greene, J. G., Porter, R. H. P., Eller, R. V., & Greenamyre, J. T. (1993) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1993.tb03634.x · Cited by 208 · see citing works

Striatal D1 and D2 dopamine receptor loss in asymptomatic mutation carriers of Huntington's disease

Weeks, R. A., Piccini, P. P., Harding, A. E., & Brooks, D. J. (1996) · Annals of Neurology · doi:10.1002/ana.410400110 · Cited by 207 · see citing works

Morphometric analysis of the prefrontal cortex in Huntington's disease

Sotrel, A., Paskevich, P. A., Kiely, D. K., Bird, E. D., Williams, R. S., & Myers, R. H. (1991) · Neurology · doi:10.1212/wnl.41.7.1117 · Cited by 204 · see citing works

STUDIES ON BRAIN BIOPSIES OF PATIENTS WITH HUNTINGTONʼS CHOREA

Tellez-Nagel, I., Johnson, T., & Terry, R. D. (1974) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-197404000-00008 · Cited by 190 · 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

Coenzyme Q₁₀ and nicotinamide block striatal lesions produced by the mitochondrial toxin malonate

Beal, M. F., Henshaw, D. R., Jenkins, B. G., Rosen, B. R., & Schulz, J. B. (1994) · Annals of Neurology · doi:10.1002/ana.410360613 · Cited by 189 · see citing works

Functional Aspects of Creatine Kinase in Brain

Hemmer, W., & Wallimann, T. (1994) · Developmental Neuroscience · doi:10.1159/000111342 · Cited by 166 · see citing works

Mechanisms underlying initiation of excitotoxicity associated with metabolic inhibition.

Zeevalk, G. D., & Nicklas, W. J. (1991) · Journal of Pharmacology and Experimental Therapeutics · doi:10.1016/s0022-3565(25)24721-8 · Cited by 164 · see citing works

Heterogeneous Topographic and Cellular Distribution of Huntingtin Expression in the Normal Human Neostriatum

Ferrante, R. J., Gutekunst, C., Persichetti, F., McNeil, S. M., Kowall, N. W., Gusella, J. F., MacDonald, M. E., Beal, M. F., & Hersch, S. M. (1997) · Journal of Neuroscience · doi:10.1523/jneurosci.17-09-03052.1997 · Cited by 162 · see citing works

3‐Nitropropionic Acid Neurotoxicity Is Attenuated in Copper/Zinc Superoxide Dismutase Transgenic Mice

Beal, M. F., Ferrante, R. J., Henshaw, R., Matthews, R. T., Chan, P. H., Kowall, N. W., Epstein, C. J., & Schulz, J. B. (1995) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.1995.65020919.x · Cited by 162 · see citing works

Mitochondrial encephalomyopathy (MELAS): Pathological study and successful therapy with coenzyme Q10 and idebenone

Ihara, Y., Namba, R., Kuroda, S., SATO, T. A., & Shirabe, T. (1989) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(89)90112-3 · Cited by 161 · see citing works

Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis

Sapp, E., Ge, P., Aizawa, H., Bird, E. D., Penney, J. B., Young, A. B., Vonsattel, J., & DiFiglia, M. (1995) · Neuroscience · doi:10.1016/0306-4522(94)00427-7 · Cited by 161 · see citing works

Neuroprotective strategies for treatment of lesions produced by mitochondrial toxins: Implications for neurodegenerative diseases

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INVOLVEMENT OF OXIDATIVE STRESS IN 3-NITROPROPIONIC ACID NEUROTOXICITY

Schulz, J. B., Henshaw, D. R., MacGarvey, U. M., & Beal, M. F. (1996) · Neurochemistry International · doi:10.1016/0197-0186(95)00122-0 · Cited by 145 · see citing works

Distribution of phosphate-activated glutaminase, succinic dehydrogenase, pyruvate dehydrogenase and γ-glutamyl transpeptidase in post-mortem brain from Huntington's disease and agonal cases

Butterworth, J. B., Yates, C. M., & Reynolds, G. P. (1985) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(85)90112-1 · Cited by 141 · see citing works

Striatal spiny neurons and cholinergic interneurons express differential ionotropic glutamatergic responses and vulnerability: Implications for ischemia and Huntington's disease

Calabresi, P., Centonze, D., Pisani, A., Sancesario, G., Gubellini, P., Marfia, G. A., & Bernardi, G. (1998) · Annals of Neurology · doi:10.1002/ana.410430506 · Cited by 133 · see citing works

Relationship between trinucliotide repeats and neuropathological changes in Huntington's diease

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