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

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

Chronic systemic pesticide exposure reproduces features of Parkinson's disease

Betarbet, R. S., Sherer, T., MacKenzie, G. M., Garcia‐Osuna, M., Panov, A. V., & Greenamyre, J. T. (2000) · Nature Neuroscience · doi:10.1038/81834 · Cited by 3,770

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

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. P., & Aronin, N. (1997) · Science · doi:10.1126/science.277.5334.1990 · Cited by 2,439

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

Mitochondrial Transport of Cations: Channels, Exchangers, and Permeability Transition

Bernardi, P. (1999) · Physiological Reviews · doi:10.1152/physrev.1999.79.4.1127 · Cited by 1,546

Aging, energy, and oxidative stress in neurodegenerative diseases

Beal, M. F. (1995) · Annals of Neurology · doi:10.1002/ana.410380304 · Cited by 1,434

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×

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

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 1,123

Interference by Huntingtin and Atrophin-1 with CBP-Mediated Transcription Leading to Cellular Toxicity

Nucifora, F. C., Sasaki, M., Peters, M. F., Huang, H., Cooper, J. K., Yamada, M., Takahashi, H., Tsuji, S., Troncoso, J. C., Dawson, V. L., et al. (2001) · Science · doi:10.1126/science.1056784 · Cited by 1,112

The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription

Steffan, J. S., Kazantsev, A. G., Spasić-Bošković, O., Greenwald, M., Zhu, Y., Goehler, H., Wanker, E. E., Bates, G. P., Housman, D. E., & Thompson, L. M. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.100110097 · Cited by 1,057

Nuclear and Neuropil Aggregates in Huntington’s Disease: Relationship to Neuropathology

Gutekunst, C.-A., Li, S.-H., Yi, H., Mulroy, J. S., Kuemmerle, S., Jones, R., Rye, D., Ferrante, R. J., Hersch, S. M., & Li, X.-J. (1999) · The Journal of Neuroscience · doi:10.1523/jneurosci.19-07-02522.1999 · Cited by 914

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 851

[42]Assessment of mitochondrial oxidative phosphorylation in patient muscle biopsies, lymphoblasts, and transmitochondrial cell lines

Trounce, I. A., Kim, Y. L., Jun, A. S., & Wallace, D. C. (1996) · Methods in enzymology on CD-ROM/Methods in enzymology · doi:10.1016/s0076-6879(96)64044-0 · Cited by 749

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

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

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

Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization

Sawa, A., Wiegand, G. W., Cooper, J. K., Margolis, R. L., Sharp, A. H., Lawler, J. F., Greenamyre, J. T., Snyder, S. H., & Ross, C. A. (1999) · Nature Medicine · doi:10.1038/13518 · Cited by 557

Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human Huntington disease gene

Cha, J.-H. J., Kosinski, C. M., Kerner, J. A., Alsdorf, S. A., Mangiarini, L., Davies, S. W., Penney, J. B., Bates, G. P., & Young, A. B. (1998) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.95.11.6480 · Cited by 504

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

Huntingtin interacts with a family of WW domain proteins

Faber, P. W. (1998) · Human Molecular Genetics · doi:10.1093/hmg/7.9.1463 · Cited by 402

Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington's disease brains, associate with membranes, and undergo calpain-dependent proteolysis

Kim, Y. J., Yi, Y., Sapp, E., Wang, Y., Cuiffo, B., Kegel, K. B., Qin, Z., D, N. A. M., & DiFiglia, M. (2001) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.221451398 · Cited by 393

Rapid Isolation of Metabolically Active Mitochondria from Rat Brain and Subregions Using Percoll Density Gradient Centrifugation

Sims, N. (1990) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.1990.tb04189.x · Cited by 388

Loss of normal huntingtin function: new developments in Huntington's disease research

Cattaneo, E. F. (2001) · Trends in Neurosciences · doi:10.1016/s0166-2236(00)01721-5 · Cited by 383

Ectopically Expressed CAG Repeats Cause Intranuclear Inclusions and a Progressive Late Onset Neurological Phenotype in the Mouse

Ordway, J. M., Tallaksen‐Greene, S. J., Gutekunst, C., Bernstein, E. M., Cearley, J. A., Wiener, H. W., Dure, L. S., Lindsey, R., Hersch, S. M., Jope, R. S., et al. (1997) · Cell · doi:10.1016/s0092-8674(00)80464-x · Cited by 370

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

HIP-I: A huntingtin interacting protein isolated by the yeast two-hybrid system

Wanker, E. E., Rovira, C., Scherzinger, E., Hasenbank, R., Walter, S., Tait, D., Colicelli, J., & Lehrach, H. (1997) · Human Molecular Genetics · doi:10.1093/hmg/6.3.487 · Cited by 260

Bioenergetics and glutamate excitotoxicity

Greene, J. G., & Greenamyre, J. T. (1996) · Progress in Neurobiology · doi:10.1016/0301-0082(96)00006-8 · Cited by 250

Wild-Type Huntingtin Reduces the Cellular Toxicity of Mutant Huntingtin In Vivo

Leavitt, B. R., Guttman, J. A., Hodgson, J., Kimel, G. H., Singaraja, R. R., Vogl, A. W., & Hayden, M. R. (2001) · The American Journal of Human Genetics · doi:10.1086/318207 · Cited by 238

The Cellular and Subcellular Localization of Huntingtin-Associated Protein 1 (HAP1): Comparison with Huntingtin in Rat and Human

Gutekunst, C., Li, S.-H., Yi, H. L., Ferrante, R. J., Li, X., & Hersch, S. M. (1998) · Journal of Neuroscience · doi:10.1523/jneurosci.18-19-07674.1998 · Cited by 181

Beyond the Qs in the polyglutamine diseases: Table 1.Preprint

Orr, H. T. (2001) · Genes & Development · doi:10.1101/gad.888401 · Cited by 168

Poly-l-Glutamine Forms Cation Channels: Relevance to the Pathogenesis of the Polyglutamine Diseases

Monoi, H., Futaki, S., Kugimiya, S., Minakata, H., & Yoshihara, K. (2000) · Biophysical Journal · doi:10.1016/s0006-3495(00)76830-5 · Cited by 86

Oligomerization of Expanded-Polyglutamine Domain Fluorescent Fusion Proteins in Cultured Mammalian Cells

Onodera, O., Burke, J. R., Miller, S., Hester, S., Tsuji, S., Roses, A. D., & Strittmatter, W. J. (1997) · Biochemical and Biophysical Research Communications · doi:10.1006/bbrc.1997.7337 · Cited by 66

Increased activity of surviving locus ceruleus neurons in Alzheimer's disease

Moser (1999) · Pure Amsterdam UMC · doi:10.1002/1531-8249(199901)45:1

Mitochondrial Dysfunction and Oxidative Stress in Neurodegenerative Diseases

Lin, M. T., & Beal, M. F. (2006) · Nature · doi:10.1038/nature05292 · Cited by 6,455

A Mitochondrial Paradigm of Metabolic and Degenerative Diseases, Aging, and Cancer: A Dawn for Evolutionary Medicine

Wallace, D. C. (2005) · Annual Review of Genetics · doi:10.1146/annurev.genet.39.110304.095751 · Cited by 3,185

Synaptic Energy Use and Supply

Harris, J. J., Jolivet, R., & Attwell, D. (2012) · Neuron · doi:10.1016/j.neuron.2012.08.019 · Cited by 1,747

Trinucleotide Repeat Disorders

Orr, H. T., & Zoghbi, H. Y. (2007) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.29.051605.113042 · Cited by 1,394

Multi-target-Directed Ligands To Combat Neurodegenerative Diseases

Cavalli, A., Bolognesi, M. L., Minarini, A., Rosini, M., Tumiatti, V., Recanatini, M., & Melchiorre, C. (2008) · Journal of Medicinal Chemistry · doi:10.1021/jm7009364 · Cited by 1,152

Role of mitochondrial dysfunction in the pathogenesis of Huntington's disease

Quintanilla, R. A., & Johnson, G. V. W. (2009) · Brain Research Bulletin · doi:10.1016/j.brainresbull.2009.07.010 · Cited by 176

In vitro effects of polyglutamine tracts on Ca2+-dependent depolarization of rat and human mitochondria: relevance to Huntington’s disease

Panov, A. V., Burke, J. R., Strittmatter, W. J., & Greenamyre, J. T. (2003) · Archives of Biochemistry and Biophysics · doi:10.1016/s0003-9861(02)00585-4 · Cited by 102

Huntington's disease, calcium, and mitochondria

Giacomello, M., Hudec, R., & Lopreiato, R. (2011) · BioFactors · doi:10.1002/biof.162 · Cited by 49

Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release

Choo, Y. S. (2004) · Human Molecular Genetics · doi:10.1093/hmg/ddh162 · Cited by 515

Mutant Huntingtin Expression Induces Mitochondrial Calcium Handling Defects in Clonal Striatal Cells

Milakovic, T., Quintanilla, R. A., & Johnson, G. V. W. (2006) · Journal of Biological Chemistry · doi:10.1074/jbc.m603845200 · Cited by 142