Synaptic dysfunction in Huntington's disease: a new perspective

Smith, R.; Brundin, P.; Li, J.-Y.

doi:10.1007/s00018-005-5084-5

Cited by 166Genetic Neurodegenerative DiseasesNeuroscience and Neuropharmacology ResearchGenetics and Neurodevelopmental Disorders

Synaptic dysfunction in Huntington's disease: a new perspective

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Smith, R., Brundin, P., Li, J.-Y.·2005·Cellular and Molecular Life Sciences

DOI 10.1007/s00018-005-5084-5PMC PMC11139070

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Abstract

Huntington's disease (HD) is caused by a polyglutamine expansion in the protein huntingtin and is characterized by intraneuronal inclusions and widespread neuronal death at the late stage of the disease. In research, most of the emphasis has been on understanding the cell death and its mechanisms. Until recently, it was believed that the vast majority, if not all, of the symptoms in HD are a direct consequence of neurodegeneration. However, increasing evidence shows that subtle alterations in synaptic function could underlie the early symptoms. It is of particular interest to understand the nature of this neuronal dysfunction. Normal huntingtin interacts with various cytoskeletal and synaptic vesicle proteins that are essential for exocytosis and endocytosis. Altered interactions of mutant huntingtin with its associated partners could contribute to abnormal synaptic transmission in HD. This review describes recent advances in understanding synaptic dysfunction in HD.

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Wild-type huntingtin has a role in vesicular trafficking and axonal transport.
“Studies suggest that wild-type huntingtin has a role in vesicular trafficking and axonal transport (Gauthier et al., 2004;Trushina et al., 2004;Smith et al., 2005a;Caviston and Holzbaur, 2009).”
From Dendritic Spine Instability Leads to Progressive Neocortical Spine Loss in a Mouse Model of Huntington's Disease (2013) · cites this paper as Smith et al., 2005a
Huntingtin aggregates could impair synaptic function, distort dendritic morphology, and disrupt trafficking and cell signaling and contribute to dendritic pathology in HD.
“These aggregates could impair synaptic function, distort dendritic morphology, and disrupt trafficking and cell signaling and contribute to the known dendritic pathology that occurs in HD (Li et al., 2003;Smith et al., 2005a).”
From Dendritic Spine Instability Leads to Progressive Neocortical Spine Loss in a Mouse Model of Huntington's Disease (2013) · cites this paper as Smith et al., 2005a

Molecular Chaperones in the Cytosol: from Nascent Chain to Folded Protein
Hartl, F. U., & Hayer-Hartl, M. K. (2002) · Science · doi:10.1126/science.1068408 · Cited by 3,335 · 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
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
Neuropathological Classification of Huntingtonʼs Disease
Vonsattel, J., Myers, R. H., STEVENS, T. J., Ferrante, R. J., Bird, E. D., & Richardson, E. P. (1985) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-198511000-00003 · Cited by 2,598 · 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
Synaptic Vesicle Phosphoproteins and Regulation of Synaptic Function
Greengard, P., Valtorta, F., Czernik, A. J., & Benfenati, F. (1993) · Science · doi:10.1126/science.8430330 · Cited by 1,311 · see citing works
Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules
Gauthier, L. R., Charrin, B. C., Borrell-Pagès, M., Dompierre, J. P., Rangone, H., Cordelières, F. P., De Mey, J., MacDonald, M. E., Leßmann, V., Humbert, S., et al. (2004) · Cell · doi:10.1016/j.cell.2004.06.018 · Cited by 1,178 · see citing works
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 1,095 · see citing works
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 · see citing works
Chaperone-Mediated Protein Folding
Fink, A. L. (1999) · Physiological Reviews · doi:10.1152/physrev.1999.79.2.425 · Cited by 1,055 · see citing works
The Ubiquitin Proteasome System in Neurodegenerative Diseases
Ciechanover, A. J., & Brundin, P. (2003) · Neuron · doi:10.1016/s0896-6273(03)00606-8 · Cited by 984 · see citing works
Self-citation
Regulation of Neurotransmitter Release by Metabotropic Glutamate Receptors
Cartmell, J., & Schoepp, D. D. (2000) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2000.0750889.x · Cited by 883 · see citing works
Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes
Nasir, J., Floresco, S., OʼKusky, J. R., Diewert, V. M., Richman, J. M., Zeisler, J., Borowski, A. H., Marth, J. D., Phillips, A. G., & Hayden, M. R. (1995) · Cell · doi:10.1016/0092-8674(95)90542-1 · Cited by 878 · 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
Huntingtin interacts with REST/NRSF to modulate the transcription of NRSE-controlled neuronal genes
Zuccato, C., Tartari, M., Crotti, A., Goffredo, D., Valenza, M., Conti, L., Cataudella, T., Leavitt, B. R., Hayden, M. R., Timmusk, T., et al. (2003) · Nature Genetics · doi:10.1038/ng1219 · Cited by 814 · see citing works
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease
Slow, E. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg169 · Cited by 799 · 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
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
Disruption of Axonal Transport by Loss of Huntingtin or Expression of Pathogenic PolyQ Proteins in Drosophila
Gunawardena, S., Her, L.-S., Brusch, R. G., Laymon, R. A., Niesman, I. R., Gordesky-Gold, B., Sintasath, L., Bonini, N. M., & Goldstein, L. S. B. (2003) · Neuron · doi:10.1016/s0896-6273(03)00594-4 · Cited by 648 · see citing works
Accumulation of Mutant Huntingtin Fragments in Aggresome-like Inclusion Bodies as a Result of Insufficient Protein Degradation
Waelter, S., Boeddrich, A., Lurz, R., Scherzinger, E., Lüder, G., Lehrach, H. R., & Wanker, E. E. (2001) · Molecular Biology of the Cell · doi:10.1091/mbc.12.5.1393 · Cited by 632 · 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
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
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
Self-citation
Competitive binding of α-actinin and calmodulin to the NMDA receptor
Wyszynski, M., Lin, J., Rao, A., Nigh, E., Beggs, A. H., Craig, A. M., & Sheng, M. (1997) · Nature · doi:10.1038/385439a0 · Cited by 596 · see citing works
Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice
Dragatsis, I., Levine, M. S., & Zeitlin, S. O. (2000) · Nature Genetics · doi:10.1038/81593 · Cited by 580 · see citing works
Huntingtin–protein interactions and the pathogenesis of Huntington's disease
Li, S.-H., & Li, X. (2004) · Trends in Genetics · doi:10.1016/j.tig.2004.01.008 · Cited by 548 · see citing works
Snares and Munc18 in synaptic vesicle fusion
Rizo, J., & Südhof, T. C. (2002) · Nature Reviews Neuroscience · doi:10.1038/nrn898 · Cited by 539 · 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
Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro
Trushina, E., Dyer, R. B., Badger, J. D., Ure, D., Eide, L., Tran, D. D., Vrieze, B. T., Legendre-Guillemin, V., McPherson, P. S., Mandavilli, B. S., et al. (2004) · Molecular and Cellular Biology · doi:10.1128/mcb.24.18.8195-8209.2004 · Cited by 520 · see citing works
Mechanisms of Synaptic Vesicle Exocytosis
Lin, R. C.-S., & Scheller, R. H. (2000) · Annual Review of Cell and Developmental Biology · doi:10.1146/annurev.cellbio.16.1.19 · Cited by 512 · see citing works
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 · see citing works
Delaying the onset of Huntington's in mice
van Dellen, A., Blakemore, C., Deacon, R., York, D. F., & Hannan, A. (2000) · Nature · doi:10.1038/35008142 · Cited by 499 · see citing works
Dynactin, a conserved, ubiquitously expressed component of an activator of vesicle motility mediated by cytoplasmic dynein.
Gill, S. R., Schroer, T. A., Szilak, I., Steuer, E. R., Sheetz, M. P., & Cleveland, D. W. (1991) · The Journal of Cell Biology · doi:10.1083/jcb.115.6.1639 · Cited by 487 · see citing works
Huntingtin and the molecular pathogenesis of Huntington's disease
Landles, C., & Bates, G. P. (2004) · EMBO Reports · doi:10.1038/sj.embor.7400250 · Cited by 485 · see citing works
Huntingtin and Huntingtin-Associated Protein 1 Influence Neuronal Calcium Signaling Mediated by Inositol-(1,4,5) Triphosphate Receptor Type 1
Tang, T.-S., Tu, H., Chan, E. Y. W., Maximov, A., Wang, Z., Wellington, C. L., Hayden, M. R., & Борисович, Б. И. (2003) · Neuron · doi:10.1016/s0896-6273(03)00366-0 · Cited by 478 · 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
Identification of potential target genes for the neuron-restrictive silencer factor.
Schoenherr, C. J., Paquette, A. J., & Anderson, D. J. (1996) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.93.18.9881 · Cited by 406 · 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
HIP1, a human homologue of S. cerevisiae Sla2p, interacts with membrane-associated huntingtin in the brain
Kalchman, M. A., Koide, H., McCutcheon, K. M., Graham, R. K., Nichol, K., Nishiyama, K., Kazemi‐Esfarjani, P., Lynn, F. C., Wellington, C. L., Metzler, M., et al. (1997) · Nature Genetics · doi:10.1038/ng0597-44 · Cited by 378 · 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
Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease
Wyttenbach, A., Carmichael, J., Swartz, J., Furlong, R. A., Narain, Y., Rankin, J., & Rubinsztein, D. C. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.97.6.2898 · Cited by 362 · 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-associated protein 1 (HAP1) interacts with the p150Glued subunit of dynactin
Engelender, S. (1997) · Human Molecular Genetics · doi:10.1093/hmg/6.13.2205 · Cited by 344 · see citing works
Interaction of Huntington Disease Protein with Transcriptional Activator Sp1
Li, S.-H., Cheng, A. L., Zhou, H., Lam, S., Rao, M., He, L., & Li, X. (2002) · Molecular and Cellular Biology · doi:10.1128/mcb.22.5.1277-1287.2002 · Cited by 343 · see citing works
HIP-I: A huntingtin interacting protein isolated by the yeast two-hybrid system
Wanker, E. E., Rovira, C., Scherzinger, E., Hasenbank, R., Wälter, S., Tait, D. S., Colicelli, J., & Lehrach, H. R. (1997) · Human Molecular Genetics · doi:10.1093/hmg/6.3.487 · Cited by 331 · see citing works
Neuropathogenic Forms of Huntingtin and Androgen Receptor Inhibit Fast Axonal Transport
Szebenyi, G., Morfini, G. A., Babcock, A., Gould, M., Selkoe, K. A., Stenoien, D. L., Young, M., Faber, P. W., MacDonald, M. E., McPhaul, M. J., et al. (2003) · Neuron · doi:10.1016/s0896-6273(03)00569-5 · Cited by 326 · see citing works
Wild-Type and Mutant Huntingtins Function in Vesicle Trafficking in the Secretory and Endocytic Pathways
Velier, J. J., Kim, M., Schwarz, C., Kim, T. W., Sapp, E., Chase, K. O., D, N. A. M., & DiFiglia, M. (1998) · Experimental Neurology · doi:10.1006/exnr.1998.6832 · Cited by 323 · see citing works
Impaired glutamate transport and glutamate-glutamine cycling: downstream effects of the Huntington mutation
Behrens, P. F. (2002) · Brain · doi:10.1093/brain/awf180 · Cited by 322 · see citing works
Impaired Glutamate Uptake in the R6 Huntington's Disease Transgenic Mice
Liévens, J.-C., Woodman, B., Mahal, A., Spasić-Bošković, O., Samuel, D., Goff, L. K., & Bates, G. P. (2001) · Neurobiology of Disease · doi:10.1006/nbdi.2001.0430 · Cited by 315 · see citing works
Mouse models of Huntington's disease
Menalled, L., & Chesselet, M. (2002) · Trends in Pharmacological Sciences · doi:10.1016/s0165-6147(00)01884-8 · Cited by 311 · see citing works
Cellular and molecular mechanisms of presynaptic assembly
Ziv, N. E., & Garner, C. C. (2004) · Nature reviews. Neuroscience · doi:10.1038/nrn1370 · Cited by 306 · 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
Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95
Sun, Y., Savanenin, A., Reddy, P. H., & Liu, Y. F. (2001) · Journal of Biological Chemistry · doi:10.1074/jbc.m103501200 · Cited by 297 · see citing works
Severe deficiencies in dopamine signaling in presymptomatic Huntington's disease mice
Bibb, J. A., Yan, Z., Svenningsson, P., Snyder, G. L., Pieribone, V. A., Horiuchi, A., Nairn, A. C., Messer, A. C., & Greengard, P. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.120166397 · Cited by 290 · 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
Interaction of Huntingtin-Associated Protein with Dynactin P150Glued
Li, S., Gutekunst, C., Hersch, S. M., & Li, X.-J. (1998) · Journal of Neuroscience · doi:10.1523/jneurosci.18-04-01261.1998 · Cited by 271 · see citing works
Orexin loss in Huntington's disease
Petersén, Å., Gil‐Mohapel, J., Maat–Schieman, M. L. C., Björkqvist, M., Tanila, H., Araújo, I. M., Smith, R. A., Popović, N., Wierup, N., Norlén, P., et al. (2004) · Human Molecular Genetics · doi:10.1093/hmg/ddi004 · Cited by 269 · see citing works
Self-citation
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
Altered neurotransmitter receptor expression in transgenic mouse models of Huntington's disease
Cha, J.-H. J., Frey, A. S., Alsdorf, S. A., Kerner, J. A., Kosinski, C. M., Mangiarini, L., Penney, J. B., Davies, S. W., Bates, G. P., & Young, A. B. (1999) · Philosophical Transactions of the Royal Society B Biological Sciences · doi:10.1098/rstb.1999.0449 · Cited by 239 · see citing works
Immunohistochemical localization of metabotropic glutamate receptors mGluR1a and mGluR2/3 in the rat basal ganglia
Testa, C. M., Friberg, I. K., Weiss, S. W., & Standaert, D. G. (1998) · The Journal of Comparative Neurology · doi:10.1002/(sici)1096-9861(19980105)390:1 · Cited by 237 · see citing works
Changes of NMDA Receptor Subunit (NR1, NR2B) and Glutamate Transporter (GLT1) mRNA Expression in Huntingtonʼs Disease—An In Situ Hybridization Study
Arzberger, T., Krampfl, K., Leimgruber, S., & Weindl, A. (1997) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-199704000-00013 · Cited by 233 · see citing works
Transgenic mice expressing a Huntington's disease mutation are resistant to quinolinic acid-induced striatal excitotoxicity
Hansson, O., Petersén, Å., Leist, M., Nicotera, P., Castilho, R. F., & Brundin, P. (1999) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.96.15.8727 · Cited by 228 · see citing works
Self-citation
Huntingtin: an iron-regulated protein essential for normal nuclear and perinuclear organelles
Hilditch-Maguire, P. (2000) · Human Molecular Genetics · doi:10.1093/hmg/9.19.2789 · Cited by 226 · see citing works
Ubiquitin-mediated sequestration of normal cellular proteins into polyglutamine aggregates
Donaldson, K. M., Li, W., Ching, K. A., Batalov, S., Tsai, C., & Joazeiro, C. A. P. (2003) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1530212100 · Cited by 225 · 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
HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis
Singaraja, R. R. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.23.2815 · Cited by 222 · see citing works
Molecular chaperones as modulators of polyglutamine protein aggregation and toxicity
Sakahira, H., Breuer, P., Hayer-Hartl, M. K., & Hartl, F. U. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.182426899 · Cited by 220 · see citing works
SH3GL3 Associates with the Huntingtin Exon 1 Protein and Promotes the Formation of Polygln-Containing Protein Aggregates
Sittler, A., Wälter, S., Wedemeyer, N., Hasenbank, R., Scherzinger, E., Eickhoff, H., Bates, G. P., Lehrach, H. R., & Wanker, E. E. (1998) · Molecular Cell · doi:10.1016/s1097-2765(00)80142-2 · Cited by 217 · see citing works
An Actin-Binding Protein of the Sla2/Huntingtin Interacting Protein 1 Family Is a Novel Component of Clathrin-Coated Pits and Vesicles
Engqvist-Goldstein, Å. E. Y., Kessels, M. M., Chopra, V. S., Hayden, M. R., & Drubin, D. G. (1999) · The Journal of Cell Biology · doi:10.1083/jcb.147.7.1503 · Cited by 213 · 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
Expanded CAG repeats in exon 1 of the Huntington's disease gene stimulate dopamine-mediated striatal neuron autophagy and degeneration
Petersén, Å. (2001) · Human Molecular Genetics · doi:10.1093/hmg/10.12.1243 · Cited by 210 · see citing works
Huntington's disease: a synaptopathy?
Li, J., Plomann, M., & Brundin, P. (2003) · Trends in Molecular Medicine · doi:10.1016/j.molmed.2003.08.006 · Cited by 208 · see citing works
Self-citation
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
Huntingtin Bodies Sequester Vesicle-Associated Proteins by a Polyproline-Dependent Interaction
Qin, Z., Wang, Y., Sapp, E., Cuiffo, B., Wanker, E. E., Hayden, M. R., Kegel, K. B., D, N. A. M., & DiFiglia, M. (2004) · Journal of Neuroscience · doi:10.1523/jneurosci.1409-03.2004 · Cited by 185 · see citing works
Perinuclear localization of huntingtin as a consequence of its binding to microtubules through an interaction with β-tubulin: relevance to Huntington's disease
Hoffner, G., Kahlem, P., & Djian, P. (2002) · Journal of Cell Science · doi:10.1242/jcs.115.5.941 · Cited by 182 · see citing works
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 · see citing works
Atrophy of the Hypothalamic Lateral Tuberal Nucleus in Huntingtonʼs Disease
Kremer, H. P. H., Roos, R. A. C., Dingjan, G. M., Bots, G. T. A. M., & Maran, E. (1990) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/00005072-199007000-00002 · Cited by 173 · see citing works
Resistance to NMDA toxicity correlates with appearance of nuclear inclusions, behavioural deficits and changes in calcium homeostasis in mice transgenic for exon 1 of the huntington gene
Hansson, O., Guatteo, E., Mercuri, N. B., Bernardi, G., Li, X., Castilho, R. F., & Brundin, P. (2001) · European Journal of Neuroscience · doi:10.1046/j.0953-816x.2001.01767.x · Cited by 154 · see citing works
Self-citation
The R6/2 transgenic mouse model of Huntington's disease develops diabetes due to deficient β-cell mass and exocytosis
Björkqvist, M., Fex, M., Renström, E., Wierup, N., Petersén, Å., Gil‐Mohapel, J., Bacos, K., Popović, N., Li, J., Sundler, F., et al. (2005) · Human Molecular Genetics · doi:10.1093/hmg/ddi053 · Cited by 143 · see citing works
Self-citation
Synapsin I is a microtubule-bundling protein
Baines, A. J., & Bennett, V. (1986) · Nature · doi:10.1038/319145a0 · Cited by 142 · see citing works
Decreased cAMP Response Element-mediated Transcription
Sugars, K. L., Brown, R. L., Cook, L. J., Swartz, J., & Rubinsztein, D. C. (2004) · Journal of Biological Chemistry · doi:10.1074/jbc.m310226200 · Cited by 139 · see citing works
Increased huntingtin protein length reduces the number of polyglutamine-induced gene expression changes in mouse models of Huntington's disease
Chan, E. Y. W. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.17.1939 · Cited by 138 · see citing works
Abnormal association of mutant huntingtin with synaptic vesicles inhibits glutamate release
Li, H. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg218 · Cited by 136 · see citing works
PACSIN 1 interacts with huntingtin and is absent from synaptic varicosities in presymptomatic Huntington's disease brains
Modregger, J. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.21.2547 · Cited by 135 · see citing works
Evidence That the Rab3a-binding Protein, Rabphilin3a, Enhances Regulated Secretion.
Chung, S.-H., Takai, Y., & Holz, R. (1995) · Journal of Biological Chemistry · doi:10.1074/jbc.270.28.16714 · Cited by 134 · see citing works
Abnormalities in the synaptic vesicle fusion machinery in Huntington’s disease
Morton, A. J., Faull, R. L. M., & Edwardson, J. M. (2001) · Brain Research Bulletin · doi:10.1016/s0361-9230(01)00611-6 · Cited by 132 · see citing works
Huntington's Disease of the Endocrine Pancreas: Insulin Deficiency and Diabetes Mellitus due to Impaired Insulin Gene Expression
Andreassen, O. A. (2002) · Neurobiology of Disease · doi:10.1006/nbdi.2002.0562 · Cited by 123 · see citing works
The hypothalamic lateral tuberal nucleus and the characteristics of neuronal loss in Huntington's disease
Kremer, H. P. H., Roos, R. A. C., Dingjan, G. M., Bots, G. T. A. M., Bruyn, G. W., & Hofman, M. A. (1991) · Neuroscience Letters · doi:10.1016/0304-3940(91)90443-w · Cited by 121 · see citing works
N-Acetylaspartate and DARPP-32 levels decrease in the corpus striatum of Huntingtonʼs disease mice
van Dellen, A., Welch, J., Dixon, R. M., Cordery, P. M., York, D. F., Styles, P., Blakemore, C. B., & Hannan, A. J. (2000) · Neuroreport · doi:10.1097/00001756-200011270-00032 · Cited by 121 · see citing works
Huntingtin inclusions do not deplete polyglutamine-containing transcription factors in HD mice
Yu, Z.-X. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.8.905 · Cited by 121 · see citing works
The role of dopamine in motor symptoms in the R6/2 transgenic mouse model of Huntington's disease
Hickey, M. A., Reynolds, G. P., & Morton, A. J. (2002) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2002.00804.x · Cited by 117 · see citing works
Physical and Functional Interaction of Rabphilin-3A with α-Actinin
Kato, M., Sasaki, T., Ohya, T., Nakanishi, H., Nishioka, H., Imamura, M., & Takai, Y. (1996) · Journal of Biological Chemistry · doi:10.1074/jbc.271.50.31775 · Cited by 113 · see citing works
Complexin II is essential for normal neurological function in mice
Glynn, D. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg249 · Cited by 108 · see citing works
Enhanced Striatal NR2B-Containing N -Methyl- d -Aspartate Receptor-Mediated Synaptic Currents in a Mouse Model of Huntington Disease
Li, L., Murphy, T. H., Hayden, M. R., & Raymond, L. A. (2004) · Journal of Neurophysiology · doi:10.1152/jn.00308.2004 · Cited by 106 · see citing works
HIP1 and HIP12 Display Differential Binding to F-actin, AP2, and Clathrin
Legendre, V., Metzler, M., Charbonneau, M., Gan, L., Chopra, V. S., Philie, J., Hayden, M. R., & McPherson, P. S. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m112310200 · Cited by 106 · see citing works
Complex alteration of NMDA receptors in transgenic Huntington's disease mouse brain: analysis of mRNA and protein expression, plasma membrane association, interacting proteins, and phosphorylation
Luthi-Carter, R., Apostol, B. L., Dunah, A. W., DeJohn, M. M., Farrell, L. A., Bates, G. P., Young, A. B., Standaert, D. G., Thompson, L. M., & Cha, J.-H. J. (2003) · Neurobiology of Disease · doi:10.1016/j.nbd.2003.08.024 · Cited by 104 · see citing works
Transcriptional dysregulation in striatal projection- and interneurons in a mouse model of Huntington's disease: neuronal selectivity and potential neuroprotective role of HAP1
Zucker, B., Luthi‐Carter, R., Kama, J. A., Dunah, A. W., Stern, E. A., Fox, J. H., Standaert, D. G., Young, A. B., & Augood, S. J. (2004) · Human Molecular Genetics · doi:10.1093/hmg/ddi014 · Cited by 99 · see citing works
CRE-Mediated Transcription Is Increased in Huntington's Disease Transgenic Mice
Obrietan, K. H., & Hoyt, K. R. (2004) · Journal of Neuroscience · doi:10.1523/jneurosci.3493-03.2004 · Cited by 98 · see citing works
Huntingtin-associated Protein 1 Interacts with Hepatocyte Growth Factor-regulated Tyrosine Kinase Substrate and Functions in Endosomal Trafficking
Li, Y., Chin, L., Levey, A. I., & Li, L. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m111612200 · Cited by 98 · see citing works
Age-dependent changes in nitric oxide synthase activity and protein expression in striata of mice transgenic for the Huntington's disease mutation.
Pérez-Severiano, F., Escalante, B., Vergara, P., Ríos, C., & Segovia, J. (2002) · Brain Research · doi:10.1016/s0006-8993(02)03102-5 · Cited by 95 · see citing works
Brain Neurotransmitter Deficits in Mice Transgenic for the Huntington’s Disease Mutation
Reynolds, G. P., Dalton, C., Tillery, C. E., Mangiarini, L., Davies, S. W., & Bates, G. P. (1999) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.1999.721773.x · Cited by 95 · see citing works
Progressive depletion of complexin II in a transgenic mouse model of Huntington's disease
Morton, A. J., & Edwardson, J. M. (2001) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2001.00059.x · Cited by 94 · see citing works
Identification of ubiquitin‐interacting proteins in purified polyglutamine aggregates
Doi, H., Mitsui, K., Kurosawa, M., Machida, Y., Kuroiwa, Y., & Nukina, N. (2004) · FEBS Letters · doi:10.1016/j.febslet.2004.06.077 · Cited by 93 · see citing works
Striatal neurochemical changes in transgenic models of Huntington's disease
Ariano, M. A., D, N. A. M., DiFiglia, M., Tagle, D. A., Sibley, D. R., Leavitt, B. R., Hayden, M. R., & Levine, M. S. (2002) · Journal of Neuroscience Research · doi:10.1002/jnr.10272 · Cited by 91 · see citing works
Evidence for Dysfunction of the Nigrostriatal Pathway in the R6/1 Line of Transgenic Huntington's Disease Mice
Petersén, Å., Puschban, Z., Lotharius, J., NicNiocaill, B., Wiekop, P., O'Connor, W. T., & Brundin, P. (2002) · Neurobiology of Disease · doi:10.1006/nbdi.2002.0534 · Cited by 90 · see citing works
Self-citation
Altered striatal amino acid neurotransmitter release monitored using microdialysis in R6/1 Huntington transgenic mice
NicNiocaill, B., Haraldsson, B., Hansson, O., O'Connor, W. T., & Brundin, P. (2001) · European Journal of Neuroscience · doi:10.1046/j.0953-816x.2000.01379.x · Cited by 90 · see citing works
Self-citation
Molecular mechanisms of neurotransmitter release
Fon, E. A., & Edwards, R. H. (2001) · Muscle & Nerve · doi:10.1002/mus.1044 · Cited by 87 · see citing works
Calcium-dependent Cleavage of Endogenous Wild-type Huntingtin in Primary Cortical Neurons
Goffredo, D., Rigamonti, D., Tartari, M., Micheli, A. D., Verderio, C., Matteoli, M., Zuccato, C., & Cattaneo, E. F. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.c200353200 · Cited by 86 · see citing works
Regulation of proteins affecting NMDA receptor-induced excitotoxicity in a Huntington's mouse model
Jarabek, B. R. (2003) · Brain · doi:10.1093/brain/awh058 · Cited by 82 · see citing works
Huntingtin protein colocalizes with lesions of neurodegenerative diseases: An investigation in Huntington's, Alzheimer's, and Pick's diseases.
Singhrao, S., Thomas, P., Wood, J. D., Macmillan, J., Neal, J. W., Harper, P. S., & Jones, A. L. (1998) · Experimental Neurology · doi:10.1006/exnr.1998.6778 · Cited by 81 · see citing works
Abnormal Phosphorylation of Synapsin I Predicts a Neuronal Transmission Impairment in the R6/2 Huntington's Disease Transgenic Mice
Liévens, J.-C. (2002) · Molecular and Cellular Neuroscience · doi:10.1006/mcne.2002.1152 · Cited by 78 · see citing works
Characterization of Endophilin B1b, a Brain-specific Membrane-associated Lysophosphatidic Acid Acyl Transferase with Properties Distinct from Endophilin A1
Modregger, J., Schmidt, A., Ritter, B., Huttner, W. Β., & Plomann, M. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.m208568200 · Cited by 71 · see citing works
Altered neuronal nitric oxide synthase expression contributes to disease progression in Huntington’s disease transgenic mice
Deckel, A. W., Tang, V., Nuttal, D., Gary, K. A., & Elder, R. F. (2002) · Brain Research · doi:10.1016/s0006-8993(02)02550-7 · Cited by 65 · see citing works
Comparative Analysis of Superoxide Dismutase Activity between Acute Pharmacological Models and a Transgenic Mouse Model of Huntington's Disease
Santamarı́a, A., Pérez‐Severiano, F., Rodrı́guez-Martı́nez, E., Maldonado, P. D., Pedraza‐Chaverrí, J., Rı́os, C., & Segovia, J. (2001) · Neurochemical Research · doi:10.1023/a:1010911417383 · Cited by 64 · see citing works
Partial resistance to malonate‐induced striatal cell death in transgenic mouse models of Huntington's disease is dependent on age and CAG repeat length
Hansson, O., Castilho, R. F., Korhonen, L., Lindholm, D., Bates, G. P., & Brundin, P. (2001) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2001.00482.x · Cited by 58 · see citing works
Self-citation
Expression of Mutant Huntingtin Blocks Exocytosis in PC12 Cells by Depletion of Complexin II
Edwardson, J. M., Wang, C., Gong, B., Wyttenbach, A., Bai, J., Jackson, M. B., Chapman, E. R., & Morton, A. J. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.m304615200 · Cited by 52 · 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
Maintenance of Susceptibility to Neurodegeneration Following Intrastriatal Injections of Quinolinic Acid in a New Transgenic Mouse Model of Huntington's Disease
Petersén, Å., Chase, K. O., Puschban, Z., DiFiglia, M., Brundin, P., & D, N. A. M. (2002) · Experimental Neurology · doi:10.1006/exnr.2002.7885 · Cited by 50 · see citing works
Self-citation
Reduced activity and protein expression of NOS in R6/2 HD transgenic mice: effects of l-NAME on symptom progression
Deckel, A. W., Gordinier, A., Nuttal, D., Tang, V., Kuwada, C. A., Freitas, R., & Gary, K. A. (2001) · Brain Research · doi:10.1016/s0006-8993(01)03000-1 · Cited by 49 · see citing works
Cysteine String Protein (CSP) Inhibition of N-type Calcium Channels Is Blocked by Mutant Huntingtin
Miller, L. C., Swayne, L. A., Chen, L., Feng, Z., Wacker, J. L., Muchowski, P. J., Zamponi, G. W., & Braun, J. E. A. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.m306230200 · Cited by 49 · see citing works
Inclusion formation in Huntington's disease R6/2 mouse muscle cultures
Orth, M., Cooper, J. A. D., Bates, G. P., & Schapira, A. H. V. (2003) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2003.02009.x · Cited by 49 · see citing works
Inhibition of tryptophan hydroxylase activity and decreased 5‐HT1A receptor binding in a mouse model of Huntington's disease
Yohrling, G. J., Jiang, G. C. ‐., DeJohn, M. M., Robertson, D. J., Vrana, K. E., & Cha, J. J. (2002) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2002.01084.x · Cited by 46 · see citing works
Expression of the Huntington's disease gene is regulated in astrocytes in the arcuate nucleus of the hypothalamus of postpartum rats
Hebb, M. O., Denovan‐Wright, E. M., & Robertson, H. A. (1999) · The FASEB Journal · doi:10.1096/fasebj.13.9.1099 · Cited by 41 · see citing works
Depletion of rabphilin 3A in a transgenic mouse model (R6/1) of Huntington's disease, a possible culprit in synaptic dysfunction
Smith, R. A., Petersén, Å., Bates, G. P., Brundin, P., & Li, J. (2005) · Neurobiology of Disease · doi:10.1016/j.nbd.2005.05.008 · Cited by 38 · see citing works
Self-citation
Transgenic mouse models of neurodegenerative disease caused by CAG/polyglutamine expansions
Bates, G. P., & Davies, S. W. (1997) · Molecular Medicine Today · doi:10.1016/s1357-4310(97)01142-8 · Cited by 32 · see citing works
Genetically confirmed clinical Huntington's disease with no observable cell loss
Caramins, M. C. (2003) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.74.7.968 · Cited by 32 · see citing works
An autopsy case with clinically and molecular genetically diagnosed Huntington's disease with only minimal non-specific neuropathological findings.
Mizuno, H., Shibayama, H., Tanaka, F., Doyu, M., Sobue, G., Iwata, H., Kobayashi, H., Yamada, K., Iwai, K., Takeuchi, T., et al. (2000) · PubMed · Cited by 29 · see citing works
Immediate‐early gene response to methamphetamine, haloperidol, and quinolinic acid is not impaired in Huntington's disease transgenic mice
MacGibbon, G. A., Hamilton, L. C., Crocker, S. F., Costain, W. J., Murphy, K. M., Robertson, H. A., & Denovan‐Wright, E. M. (2002) · Journal of Neuroscience Research · doi:10.1002/jnr.10100 · Cited by 23 · see citing works

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Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease
Zuccato, C., Valenza, M., & Cattaneo, E. (2010) · Physiological Reviews · doi:10.1152/physrev.00041.2009 · Cited by 848 · see citing works
Normal huntingtin function: an alternative approach to Huntington's disease
Cattaneo, E. F., Zuccato, C., & Tartari, M. (2005) · Nature reviews. Neuroscience · doi:10.1038/nrn1806 · Cited by 655 · see citing works
The role of glutamate transporters in neurodegenerative diseases and potential opportunities for intervention
Sheldon, A. L., & Robinson, M. B. (2007) · Neurochemistry International · doi:10.1016/j.neuint.2007.03.012 · Cited by 593 · see citing works
Localization of LRRK2 to membranous and vesicular structures in mammalian brain
Biskup, S., Moore, D. J., Celsi, F., Higashi, S., West, A. B., Andrabi, S. A., Kurkinen, K. M. A., Yu, S., Savitt, J. M., Waldvogel, H. J., et al. (2006) · Annals of Neurology · doi:10.1002/ana.21019 · Cited by 511 · see citing works
Mechanisms of neurodegeneration in Huntington’s disease
Gil‐Mohapel, J., & Rego, A. C. (2008) · European Journal of Neuroscience · doi:10.1111/j.1460-9568.2008.06310.x · Cited by 480 · see citing works
Adenosine receptors and brain diseases: neuroprotection and neurodegeneration
Gomes, C. V., Kaster, M. P., Tomé, A. R., Agostinho, P. M., & Cunha, R. A. (2011) · Biochimica et Biophysica Acta (BBA) - Biomembranes · doi:10.1016/j.bbamem.2010.12.001 · Cited by 419 · 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
The corticostriatal pathway in Huntington's disease
Cepeda, C., Wu, N.-P., André, V. M., Cummings, D. M., & LEVINE, M. (2006) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2006.11.001 · Cited by 309 · see citing works
Early synaptic pathophysiology in neurodegeneration: insights from Huntington's disease
Milnerwood, A. J., & Raymond, L. A. (2010) · Trends in Neurosciences · doi:10.1016/j.tins.2010.08.002 · Cited by 311 · see citing works
Axon diodes for the reconstruction of oriented neuronal networks in microfluidic chambers
Peyrin, J., Deleglise, B., Saïas, L., Vignes, M., Gougis, P., Magnifico, S., Bétuing, S., Pietri, M., Caboche, J., Vanhoutte, P., et al. (2011) · Lab on a Chip · doi:10.1039/c1lc20014c · Cited by 270 · see citing works
Delivery of GABAARs to Synapses Is Mediated by HAP1-KIF5 and Disrupted by Mutant Huntingtin
Twelvetrees, A. E., Yuen, E. Y., Arancibia‐Cárcamo, I. L., MacAskill, A. F., Rostaing, P., Lumb, M. J., Humbert, S., Triller, A., Saudou, F., Yan, Z., et al. (2010) · Neuron · doi:10.1016/j.neuron.2009.12.007 · Cited by 276 · see citing works
The biological function of the Huntingtin protein and its relevance to Huntington's Disease pathology.
Schulte, J., & Littleton, J. T. (2011) · PubMed · Cited by 203 · 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
Suppression of Neurodegeneration and Increased Neurotransmission Caused by Expanded Full-Length Huntingtin Accumulating in the Cytoplasm
Romero, E., Cha, G., Verstreken, P., Ly, C. V., Hughes, R. E., Bellen, H. J., & Botas, J. (2008) · Neuron · doi:10.1016/j.neuron.2007.11.025 · Cited by 171 · see citing works
Age-Dependent Alterations of Corticostriatal Activity in the YAC128 Mouse Model of Huntington Disease
Joshi, P. R., Wu, N.-P., André, V. M., Cummings, D. M., Cepeda, C., Joyce, J. A., Carroll, J. B., Leavitt, B. R., Hayden, M. R., Levine, M. S., et al. (2009) · Journal of Neuroscience · doi:10.1523/jneurosci.5687-08.2009 · Cited by 166 · see citing works
Impaired ubiquitin–proteasome system activity in the synapses of Huntington's disease mice
Wang, J., Wang, C.-E., Orr, A. L., Tydlacka, S., Li, S., & Li, X. (2008) · The Journal of Cell Biology · doi:10.1083/jcb.200709080 · Cited by 168 · see citing works
Cholesterol Defect Is Marked across Multiple Rodent Models of Huntington's Disease and Is Manifest in Astrocytes
Valenza, M., Leoni, V., Karasinska, J. M., Petricca, L., Fan, J., Carroll, J., Pouladi, M. A., Fossale, E., Nguyen, H. P., Riess, O., et al. (2010) · The Journal of Neuroscience · doi:10.1523/jneurosci.0917-10.2010 · Cited by 166 · see citing works
Morphogenesis of the Endoplasmic Reticulum: Beyond Active Membrane Expansion
Vedrenne, C., & Hauri, H. (2006) · Traffic · doi:10.1111/j.1600-0854.2006.00419.x · Cited by 151 · see citing works
Differential ubiquitination and degradation of huntingtin fragments modulated by ubiquitin-protein ligase E3A
Bhat, K. P., Yan, S., Wang, C.-E., Li, S., & Li, X. (2014) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1402215111 · Cited by 142 · see citing works
A Signaling Cascade of Nuclear Calcium-CREB-ATF3 Activated by Synaptic NMDA Receptors Defines a Gene Repression Module That Protects against Extrasynaptic NMDA Receptor-Induced Neuronal Cell Death and Ischemic Brain Damage
Zhang, S., Buchthal, B., Lau, D., Hayer, S. N., Dick, O., Schwaninger, M., Veltkamp, R. C., Zou, M., Weiss, U. K., & Bading, H. (2011) · Journal of Neuroscience · doi:10.1523/jneurosci.2672-10.2011 · Cited by 137 · see citing works
The Role of Microglia in Synaptic Stripping and Synaptic Degeneration: A Revised Perspective
Perry, V. H., & O'Connor, V. M. (2010) · ASN NEURO · doi:10.1042/an20100024 · Cited by 131 · see citing works
The Structure and Function of Endophilin Proteins
Kjærulff, O., Brodin, L., & Jung, A. G. (2010) · Cell Biochemistry and Biophysics · doi:10.1007/s12013-010-9137-5 · Cited by 128 · see citing works
The bile acid TUDCA and neurodegenerative disorders: An overview
Zangerolamo, L., Vettorazzi, J. F., Rosa, L. R. D. O., Carneiro, E. M., & Barbosa, H. C. L. (2021) · Life Sciences · doi:10.1016/j.lfs.2021.119252 · Cited by 127 · see citing works
Pathophysiology of Huntingtonʼs disease: from huntingtin functions to potential treatments
Roze, E. F., Saudou, F., & Caboche, J. (2008) · Current Opinion in Neurology · doi:10.1097/wco.0b013e328304b692 · Cited by 127 · see citing works
Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals, contributing to neuropathology in humans and mice
Kim, S., Zhang, Z., Sarkar, C., Tsai, P., Lee, Y., Dye, L., & Mukherjee, A. B. (2008) · Journal of Clinical Investigation · doi:10.1172/jci33482 · Cited by 126 · see citing works
A Time Course Analysis of the Electrophysiological Properties of Neurons Differentiated from Human Induced Pluripotent Stem Cells (iPSCs)
Prè, D., Nestor, M. W., Sproul, A. A., Jacob, S., Koppensteiner, P., Chinchalongporn, V., Zimmer, M., Yamamoto, A., Noggle, S., & Arancio, O. (2014) · PLoS ONE · doi:10.1371/journal.pone.0103418 · Cited by 124 · see citing works
Molecular Pathophysiological Mechanisms in Huntington’s Disease
Jurcău, A. (2022) · Biomedicines · doi:10.3390/biomedicines10061432 · Cited by 111 · see citing works
Synapse elimination during development and disease: immune molecules take centre stage
Schafer, D. P., & Stevens, B. (2010) · Biochemical Society Transactions · doi:10.1042/bst0380476 · Cited by 110 · see citing works
Dendritic Spine Instability Leads to Progressive Neocortical Spine Loss in a Mouse Model of Huntington's Disease
Murmu, R. P., Li, W., Holtmaat, A., & Li, J.-Y. (2013) · Journal of Neuroscience · doi:10.1523/jneurosci.5284-12.2013 · Cited by 95 · see citing works
“…in synaptic transmission, such as the scaffolding protein PSD-95, and AMPA receptors among many others and lead to depletion of these proteins at their site of action (Cha et al., 1998; Sun et al., 2001; Luthi-Carter et al., 2003; Smith et al., 2005a,b, 2007; Nithianantharajah and Hannan, 2012).”
BackgroundSelf-citation
Huntington Disease and the Huntingtin Protein
Zheng, Z., & Diamond, M. I. (2012) · Progress in molecular biology and translational science · doi:10.1016/b978-0-12-385883-2.00010-2 · Cited by 104 · see citing works
An in vitro perspective on the molecular mechanisms underlying mutant huntingtin protein toxicity
Cisbani, G., & Cicchetti, F. (2012) · Cell Death and Disease · doi:10.1038/cddis.2012.121 · Cited by 102 · see citing works
Wild-type HTT modulates the enzymatic activity of the neuronal palmitoyl transferase HIP14
Huang, K., Sanders, S. S., Kang, R., Carroll, J. B., Sutton, L. M., Wan, J., Singaraja, R. R., Young, F. B. J., Liu, L., El-Husseini, A., et al. (2011) · Human Molecular Genetics · doi:10.1093/hmg/ddr242 · Cited by 97 · see citing works
D-β-Hydroxybutyrate Is Protective in Mouse Models of Huntington's Disease
Lim, S., Chesser, A. S., Grima, J. C., Rappold, P. M., Blum, D. L., Przedborski, S., & Tieu, K. (2011) · PLoS ONE · doi:10.1371/journal.pone.0024620 · Cited by 94 · see citing works
Neurogranin: A Potential Biomarker of Neurological and Mental Diseases
Xiang, Y., Xin, J., Le, W., & Yang, Y. (2020) · Frontiers in Aging Neuroscience · doi:10.3389/fnagi.2020.584743 · Cited by 96 · see citing works
The Influence of Na+, K+-ATPase on Glutamate Signaling in Neurodegenerative Diseases and Senescence
Kinoshita, P. F., Leite, J. A., Orellana, A. M., Vasconcelos, A. R., Quintas, L. E. M., Kawamoto, E. M., & Scavone, C. (2016) · Frontiers in Physiology · doi:10.3389/fphys.2016.00195 · Cited by 91 · see citing works
Activated microglia proliferate at neurites of mutant huntingtin-expressing neurons
Kraft, A. D., Kaltenbach, L. S., Lo, D. C., & Harry, G. J. (2011) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2011.02.015 · Cited by 90 · see citing works
Loss of SNAP‐25 and rabphilin 3a in sensory‐motor cortex in Huntington’s disease
Smith, R. A., Klein, P., Koc‐Schmitz, Y., Waldvogel, H. J., Faull, R. L. M., Brundin, P., Plomann, M., & Li, J. (2007) · Journal of Neurochemistry · doi:10.1111/j.1471-4159.2007.04703.x · Cited by 87 · see citing works
Self-citation
Is the ubiquitin-proteasome system impaired in Huntington’s disease?
Ortega, Z., Dı́az-Hernández, M., & Lucas, J. J. (2007) · Cellular and Molecular Life Sciences · doi:10.1007/s00018-007-7222-8 · Cited by 85 · see citing works
Synaptic Activity-Mediated Suppression of p53 and Induction of Nuclear Calcium-Regulated Neuroprotective Genes Promote Survival through Inhibition of Mitochondrial Permeability Transition
Lau, D., & Bading, H. (2009) · Journal of Neuroscience · doi:10.1523/jneurosci.0802-09.2009 · Cited by 82 · see citing works
Cognitive Dysfunction in Huntington's Disease: Humans, Mouse Models and Molecular Mechanisms
Giralt, A., Saavedra, A., Alberch, J., & Pérez‐Navarro, E. (2012) · Journal of Huntington s Disease · doi:10.3233/jhd-120023 · Cited by 76 · see citing works
Antisense Therapy in Neurology
Lee, J. K., & Yokota, T. (2013) · Journal of Personalized Medicine · doi:10.3390/jpm3030144 · Cited by 74 · see citing works
Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases
Branco, J., Al‐Ramahi, I., Ukani, L., Perez, A. M., Fernández-Fúnez, P., Rincón-Limas, D. E., & Botas, J. (2007) · Human Molecular Genetics · doi:10.1093/hmg/ddm315 · Cited by 74 · see citing works
Rab11 rescues synaptic dysfunction and behavioural deficits in a Drosophila model of Huntington's disease
Steinert, J. R., Campesan, S., Richards, P., Kyriacou, C. P., Forsythe, I. D., & Giorgini, F. (2012) · Human Molecular Genetics · doi:10.1093/hmg/dds117 · Cited by 75 · see citing works
Synaptic Dysfunction in Huntington’s Disease: Lessons from Genetic Animal Models
Cepeda, C., & Levine, M. S. (2020) · The Neuroscientist · doi:10.1177/1073858420972662 · Cited by 68 · see citing works
When intracellular logistics fails - genetic defects in membrane trafficking
Olkkonen, V. M., & Ikonen, E. (2006) · Journal of Cell Science · doi:10.1242/jcs.03303 · Cited by 70 · see citing works
Impaired α-Amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) Receptor Trafficking and Function by Mutant Huntingtin
Mandal, M., Jing, W., Zhong, P., Cheng, J., Duffney, L. J., Liu, W., Yuen, E. Y., Twelvetrees, A. E., Li, S., Li, X., et al. (2011) · Journal of Biological Chemistry · doi:10.1074/jbc.m111.236521 · Cited by 60 · see citing works
Increased Neurotransmitter Release at the Neuromuscular Junction in a Mouse Model of Polyglutamine Disease
Rozas, J. L., Gómez‐Sánchez, L., Tomás‐Zapico, C., Lucas, J. J., & Fernández‐Chacón, R. (2011) · Journal of Neuroscience · doi:10.1523/jneurosci.2011-10.2011 · Cited by 59 · see citing works
Large-scale functional RNAi screen in C. elegans identifies genes that regulate the dysfunction of mutant polyglutamine neurons
Lejeune, F., Mesrob, L., Parmentier, F., Bicep, C., Vázquez‐Manrique, R. P., Parker, J. A., Vert, J., Tourette, C., & Néri, C. (2012) · BMC Genomics · doi:10.1186/1471-2164-13-91 · Cited by 59 · see citing works
Synaptopathic mechanisms of neurodegeneration and dementia: Insights from Huntington’s disease
Tyebji, S., & Hannan, A. J. (2017) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2017.03.008 · Cited by 56 · see citing works
Metabolite mapping reveals severe widespread perturbation of multiple metabolic processes in Huntington's disease human brain
Patassini, S., Begley, P., Xu, J., Church, S. J., Reid, S. J., Kim, E. H., Curtis, M. A., Dragunow, M., Waldvogel, H. J., Snell, R. G., et al. (2016) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2016.06.002 · Cited by 56 · see citing works
Axonopathy in Huntington's disease
Li, J.-Y., & Conforti, L. (2013) · Experimental Neurology · doi:10.1016/j.expneurol.2012.08.010 · Cited by 55 · see citing works
Self-citation
Integrative hypothesis for Huntington’s disease: a brief review on experimental evidence.
de la Cruz, V. P., & Santamarı́a, A. (2007) · Physiological Research · doi:10.33549/physiolres.931049 · Cited by 54 · see citing works
Polyglutamine gene function and dysfunction in the ageing brain
Hands, S. L., Sinadinos, C., & Wyttenbach, A. (2008) · Biochimica et Biophysica Acta (BBA) - Gene Regulatory Mechanisms · doi:10.1016/j.bbagrm.2008.05.008 · Cited by 54 · see citing works
Huntington’s Disease
Roze, E. F., Bonnet, C., Bétuing, S., & Caboche, J. (2010) · Advances in experimental medicine and biology · doi:10.1007/978-1-4419-6448-9_5 · Cited by 53 · see citing works
Memory and synaptic deficits in Hip14/DHHC17 knockout mice
Milnerwood, A. J., Parsons, M. P., Young, F. B. J., Singaraja, R. R., Franciosi, S., Volta, M., Bergeron, S., Hayden, M. R., & Raymond, L. A. (2013) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1222384110 · Cited by 52 · see citing works
Dysfunction of the SNARE complex in neurological and psychiatric disorders
Chen, F., Chen, H., Chen, Y., Wei, W., Sun, Y., Zhang, L., Cui, L., & Wang, Y. (2021) · Pharmacological Research · doi:10.1016/j.phrs.2021.105469 · Cited by 52 · see citing works
High resolution time-course mapping of early transcriptomic, molecular and cellular phenotypes in Huntington’s disease CAG knock-in mice across multiple genetic backgrounds
Ament, S. A., Pearl, J. R., Grindeland, A., Claire, J. S., Earls, J. C., Kovalenko, M. V., Gillis, T., Mysore, J. S., Gusella, J. F., Lee, J., et al. (2017) · Human Molecular Genetics · doi:10.1093/hmg/ddx006 · Cited by 50 · see citing works
Dysregulation of Corticostriatal Connectivity in Huntington’s Disease: A Role for Dopamine Modulation
Rangel‐Barajas, C., & Rebec, G. V. (2016) · Journal of Huntington s Disease · doi:10.3233/jhd-160221 · Cited by 49 · see citing works
Huntington's disease, calcium, and mitochondria
Giacomello, M., Hudec, R., & Lopreiato, R. (2011) · BioFactors · doi:10.1002/biof.162 · Cited by 47 · see citing works
Platelet abnormalities in Huntington’s disease
Denis, H. L., Lamontagne‐Proulx, J., St‐Amour, I., Mason, S., Rowley, J. W., Cloutier, N., Tremblay, M., Vincent, A. T., Gould, P. V., Chouinard, S., et al. (2018) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2018-318854 · Cited by 47 · see citing works
In Situ Mitochondrial Ca2+ Buffering Differences of Intact Neurons and Astrocytes from Cortex and Striatum
Oliveira, J. M. A., & Gonçalves, J. (2008) · Journal of Biological Chemistry · doi:10.1074/jbc.m807459200 · Cited by 45 · see citing works
Developmental NMDA receptor dysregulation in the infantile neuronal ceroid lipofuscinosis mouse model
Koster, K. P., Francesconi, W. G., Berton, F., Alahmadi, S., Srinivas, R., & Yoshii, A. (2019) · eLife · doi:10.7554/elife.40316 · Cited by 41 · see citing works
Syntaxin 1A promotes the endocytic sorting of EAAC1 leading to inhibition of glutamate transport
Yu, Y., Li, S., Xia, P., Tang, Y., Bao, L., & Pei, G. (2006) · Journal of Cell Science · doi:10.1242/jcs.03151 · Cited by 42 · see citing works
Genetic Modifiers of Drosophila Palmitoyl–Protein Thioesterase 1-Induced Degeneration
Buff, H., Smith, A. C., & Korey, C. A. (2007) · Genetics · doi:10.1534/genetics.106.067983 · Cited by 40 · see citing works
Nmnat restores neuronal integrity by neutralizing mutant Huntingtin aggregate-induced progressive toxicity
Zhu, Y., Li, C. V., Tao, X., Brazill, J. M., Park, J., Diaz-Perez, Z., & Zhai, R. G. (2019) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1904563116 · Cited by 41 · see citing works
Azadiradione Restores Protein Quality Control and Ameliorates the Disease Pathogenesis in a Mouse Model of Huntington’s Disease
Singh, B. K., Vatsa, N., Nelson, V. K., Kumar, V., Kumar, R., Mandal, S. C., Pal, M., & Jana, N. R. (2018) · Molecular Neurobiology · doi:10.1007/s12035-017-0853-3 · Cited by 40 · see citing works
Synaptic rearrangement following axonal injury: Old and new players
Spejo, A. B., & de Oliveira, A. L. R. (2014) · Neuropharmacology · doi:10.1016/j.neuropharm.2014.11.002 · Cited by 40 · see citing works
Cognitive dysfunction in H untington's disease: mechanisms and therapeutic strategies beyond BDNF
Puigdellívol, M., Saavedra, A., & Pérez‐Navarro, E. (2016) · Brain Pathology · doi:10.1111/bpa.12432 · Cited by 37 · see citing works
The Evidence for the Spread and Seeding Capacities of the Mutant Huntingtin Protein in in Vitro Systems and Their Therapeutic Implications
Masnata, M., & Cicchetti, F. (2017) · Frontiers in Neuroscience · doi:10.3389/fnins.2017.00647 · Cited by 37 · see citing works
Synaptic Communication Engineering for Future Cognitive Brain–Machine Interfaces
Veletić, M., & Balasingham, I. (2019) · Proceedings of the IEEE · doi:10.1109/jproc.2019.2915199 · Cited by 39 · see citing works
Striatal Synaptosomes from Hdh140Q/140Q Knock-in Mice have Altered Protein Levels, Novel Sites of Methionine Oxidation, and Excess Glutamate Release after Stimulation
Valencia, A., Sapp, E., Kimm, J. S., McClory, H., Ansong, K. A., Yohrling, G. J., Kwak, S., Kegel, K. B., Green, K. M., Shaffer, S. A., et al. (2013) · Journal of Huntington s Disease · doi:10.3233/jhd-130080 · Cited by 39 · see citing works
Disrupted GABAAR trafficking and synaptic inhibition in a mouse model of Huntington's disease
Yuen, E. Y., Wei, J., Zhong, P., & Yan, Z. (2012) · Neurobiology of Disease · doi:10.1016/j.nbd.2012.02.015 · Cited by 38 · see citing works
Autophagy--a basic mechanism and a potential role for neurodegeneration.
Bursch, W., & Ellinger, A. (2005) · PubMed · Cited by 37 · see citing works
Discriminative behavioral assessment unveils remarkable reactive astrocytosis and early molecular correlates in basal ganglia of 3-nitropropionic acid subchronic treated rats
Cirillo, G., Maggio, N., Bianco, M. R., Vollono, C., Sellitti, S., & Papa, M. (2009) · Neurochemistry International · doi:10.1016/j.neuint.2009.09.013 · Cited by 36 · see citing works
Alteration in Synaptic Junction Proteins following Traumatic Brain Injury
Merlo, L., Cimino, F., Angileri, F. F., Torre, D. L., Conti, A., Cardali, S. M., Saija, A., & Germanò, A. (2014) · Journal of Neurotrauma · doi:10.1089/neu.2014.3385 · Cited by 36 · see citing works
Age-related axonal swellings precede other neuropathological hallmarks in a knock-in mouse model of Huntington's disease
Marangoni, M., Adalbert, R., Janečková, L., Patrick, J., Kohli, J. S., Coleman, M. P., & Conforti, L. (2014) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2014.04.024 · Cited by 35 · see citing works
Synaptic mutant huntingtin inhibits synapsin-1 phosphorylation and causes neurological symptoms
Xu, Q., Huang, S., Song, M., Wang, C.-E., Yan, S., Liu, X., Gaertig, M. A., Yu, S. P., Li, H., Li, S., et al. (2013) · The Journal of Cell Biology · doi:10.1083/jcb.201303146 · Cited by 35 · see citing works
Stem cell transplantation for Huntington’s diseases
Choi, K., Choi, Y., & Hong, S. (2017) · Methods · doi:10.1016/j.ymeth.2017.08.017 · Cited by 34 · see citing works
Impaired development of cortico-striatal synaptic connectivity in a cell culture model of Huntington's disease
Buren, C., Parsons, M. P., Smith-Dijak, A., & Raymond, L. A. (2015) · Neurobiology of Disease · doi:10.1016/j.nbd.2015.12.009 · Cited by 33 · see citing works
Cerebrospinal fluid neurogranin and TREM2 in Huntington’s disease
Byrne, L. M., Rodrigues, F. B., Johnson, E. B., Vita, E. D., Blennow, K., Scahill, R. I., Zetterberg, H., Heslegrave, A., & Wild, E. J. (2018) · Scientific Reports · doi:10.1038/s41598-018-21788-x · Cited by 31 · see citing works
Sp1 Regulates Human Huntingtin Gene Expression
Wang, R., Luo, Y., Ly, P. T. T., Cai, F., Zhou, W., Zou, H., & Song, W. (2012) · Journal of Molecular Neuroscience · doi:10.1007/s12031-012-9739-z · Cited by 33 · see citing works
Sleep/Wake Physiology and Quantitative Electroencephalogram Analysis of the Neuroligin-3 Knockout Rat Model of Autism Spectrum Disorder
Thomas, A. M., Schwartz, M. D., Saxe, M., & Kilduff, T. S. (2017) · SLEEP · doi:10.1093/sleep/zsx138 · Cited by 32 · see citing works
Investigating the Transition of Pre-Symptomatic to Symptomatic Huntington’s Disease Status Based on Omics Data
Christodoulou, C. C., Zachariou, M., Tomazou, M., Karatzas, E., Demetriou, C. A., Zamba‐Papanicolaou, E., & Spyrou, G. M. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21197414 · Cited by 31 · see citing works
Glutamate‐induced alterations in Ca2+ signaling are modulated by mitochondrial Ca2+ handling capacity in brain slices of R6/1 transgenic mice
Rosenstock, T. R., Bertoncini, C. R. A., Teles, A. V. F. F., Hirata, H., Fernandes, M. J. D. S., & Smaili, S. S. (2010) · European Journal of Neuroscience · doi:10.1111/j.1460-9568.2010.07268.x · Cited by 30 · see citing works
Immunohistochemical analysis of huntingtin-associated protein 1 in adult rat spinal cord and its regional relationship with androgen receptor
Islam, M. N., Takeshita, Y., Yanai, A., Amami, I., Jahan, M. R., Wroblewski, G., Nemoto, J., Fujinaga, R., & Shinoda, K. (2016) · Neuroscience · doi:10.1016/j.neuroscience.2016.10.053 · Cited by 30 · see citing works
Altered Synaptic Vesicle Release and Ca2+ Influx at Single Presynaptic Terminals of Cortical Neurons in a Knock-in Mouse Model of Huntington’s Disease
Chen, S., Yu, C., Rong, L., Li, C. H., Qin, X., Ryu, H., & Park, H. (2018) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2018.00478 · Cited by 28 · see citing works
Presynaptic dysfunction in Huntington's disease
Rozas, J. L., Gómez‐Sánchez, L., Tomás‐Zapico, C., Lucas, J. J., & Fernández‐Chacón, R. (2010) · Biochemical Society Transactions · doi:10.1042/bst0380488 · Cited by 29 · see citing works
Targeting the Cholinergic System to Develop a Novel Therapy for Huntington’s Disease
D’Souza, G. X., & Waldvogel, H. J. (2016) · Journal of Huntington s Disease · doi:10.3233/jhd-160200 · Cited by 28 · see citing works
Neurotrophic Enhancers as Therapy for Behavioral Deficits in Rodent Models of Huntington's Disease: Use of Gangliosides, Substituted Pyrimidines, and Mesenchymal Stem Cells
Dunbar, G. L., Sandstrom, M. I., Rossignol, J., & Lescaudron, L. (2006) · Behavioral and Cognitive Neuroscience Reviews · doi:10.1177/1534582306289367 · Cited by 28 · see citing works
Electroconvulsive shock ameliorates disease processes and extends survival in huntingtin mutant mice
Mughal, M. R., Baharani, A., Chigurupati, S., Son, T. G., Chen, E., Yang, P. H., Okun, E., Arumugam, T. V., Chan, S. L., & Mattson, M. P. (2010) · Human Molecular Genetics · doi:10.1093/hmg/ddq512 · Cited by 28 · see citing works
Modulation of nuclear REST by alternative splicing: a potential therapeutic target for Huntington's disease
Chen, G., Ma, Q., Goswami, D. B., Shang, J., & Miller, G. M. (2017) · Journal of Cellular and Molecular Medicine · doi:10.1111/jcmm.13209 · Cited by 27 · see citing works
Cdk5 Contributes to Huntington’s Disease Learning and Memory Deficits via Modulation of Brain Region-Specific Substrates
Alvarez-Periel, E., Puigdellívol, M., Brito, V., Plattner, F., Bibb, J. A., Alberch, J., & Ginés, S. (2017) · Molecular Neurobiology · doi:10.1007/s12035-017-0828-4 · Cited by 27 · see citing works
Early Down-Regulation of PKCδ as a Pro-Survival Mechanism in Huntington’s Disease
Rué, L., Alcalá‐Vida, R., López-Soop, G., Creus‐Muncunill, J., Alberch, J., & Pérez‐Navarro, E. (2013) · NeuroMolecular Medicine · doi:10.1007/s12017-013-8248-8 · Cited by 26 · see citing works
Selective Vulnerability of Basal Ganglia: Insights into the Mechanisms of Bilateral Striatal Necrosis
Cirillo, G., Cirillo, M., Panetsos, F., Virtuoso, A., & Papa, M. (2019) · Journal of Neuropathology & Experimental Neurology · doi:10.1093/jnen/nly123 · Cited by 26 · see citing works
Muscle atrophy is associated with cervical spinal motoneuron loss in BACHD mouse model for Huntington's disease
Valadão, P. A. C., de Aragão, B. C., Andrade, J. N., Magalhães-Gomes, M. P. S., Foureaux, G., Joviano‐Santos, J. V., Nogueira, J. C., Ribeiro, F. M., Tapia, J. C., & Guatimosim, C. (2016) · European Journal of Neuroscience · doi:10.1111/ejn.13510 · Cited by 23 · see citing works
Solid Lipid Curcumin Particles Protect Medium Spiny Neuronal Morphology, and Reduce Learning and Memory Deficits in the YAC128 Mouse Model of Huntington’s Disease
Gharaibeh, A., Maiti, P., Culver, R., Heileman, S., Srinageshwar, B., Story, D. T., Spelde, K., Paladugu, L., Munro, N., Muhn, N., et al. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21249542 · Cited by 24 · see citing works
Expression of huntingtin-associated protein 1 in adult mouse dorsal root ganglia and its neurochemical characterization in reference to sensory neuron subpopulations
Islam, M. N., Maeda, N., Miyasato, E., Jahan, M. R., Tarif, A. M. M., Ishino, T., Nozaki, K., Masumoto, K., Yanai, A., & Shinoda, K. (2020) · IBRO Reports · doi:10.1016/j.ibror.2020.10.001 · Cited by 23 · see citing works
Calpastatin ablation aggravates the molecular phenotype in cell and animal models of Huntington disease
Weber, J. J., Kloock, S., Nagel, M., Ortiz-Rios, M. M., Hofmann, J. I., Rieß, O., & Nguyen, H. P. (2018) · Neuropharmacology · doi:10.1016/j.neuropharm.2018.01.022 · Cited by 21 · see citing works
Protein homeostasis in neurons and its pathological alterations
Malgaroli, A., Vallar, L., & Zimarino, V. (2006) · Current Opinion in Neurobiology · doi:10.1016/j.conb.2006.05.009 · Cited by 21 · see citing works
Microcirculation response to local cooling in patients with Huntington’s disease
Melik, Ž., Kobal, J., Cankar, K., & Štrucl, M. (2011) · Journal of Neurology · doi:10.1007/s00415-011-6279-3 · Cited by 20 · see citing works

Huntington's disease: a synaptopathy?
Li, J., Plomann, M., & Brundin, P. (2003) · Trends in Molecular Medicine · doi:10.1016/j.molmed.2003.08.006 · Cited by 208 · see citing works
Self-citation
Synaptic Abnormalities Associated with Huntington’s Disease
Milnerwood, A. J., & Raymond, L. A. (2006) · Springer eBooks · doi:10.1007/978-0-387-32562-0_32 · Cited by 7 · see citing works
Presynaptic dysfunction in Huntington's disease
Rozas, J. L., Gómez‐Sánchez, L., Tomás‐Zapico, C., Lucas, J. J., & Fernández‐Chacón, R. (2010) · Biochemical Society Transactions · doi:10.1042/bst0380488 · Cited by 29 · see citing works
Synaptic Dysfunction in Huntington’s Disease
Glynn, D., & Morton, A. J. (2010) · Springer eBooks · doi:10.1007/978-1-4419-7061-9_12
Abnormal association of mutant huntingtin with synaptic vesicles inhibits glutamate release
Li, H. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg218 · Cited by 136 · see citing works

Authors: Smith, R., Brundin, P., & Li, J.-Y.
Affiliations: Lund University (SE)
Corresponding author: Li, J.-Y.
ORCID: Smith, R.
Brundin, P.
Li, J.-Y.
Venue: Cellular and Molecular Life Sciences
Publisher: Springer Science and Business Media LLC
Published: 2005-06-15
Volume: 62
Issue: 17
Pages: 1901-1912
ISSN: 1420-682X (ISSN-L)
1420-9071
DOI: 10.1007/s00018-005-5084-5
PMC: PMC11139070
OpenAlex: W2132164151
Cited by: 166
References: 132
Citations by year: 2026: 2 · 2025: 3 · 2023: 4 · 2022: 5 · 2021: 7 · 2020: 8 · 2019: 4 · 2018: 10 · 2017: 8 · 2016: 10 · 2015: 4 · 2014: 10 · 2013: 10 · 2012: 12
FWCI: 3.87
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Licence: www.springer.com/tdm (tdm)
Locations: Cellular and Molecular Life Sciences (publishedVersion)
PubMed Central (publishedVersion)
PubMed (publishedVersion)
Lund University Publications (Lund University) (submittedVersion)
Topics: Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Neuroscience and Neuropharmacology Research
Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
Keywords: Huntington's disease, synaptic dysfunction, huntingtin, polyglutamine expansion, mutant huntingtin, synaptic vesicle proteins, exocytosis, endocytosis, synaptic transmission, neurodegeneration, neuronal dysfunction
MeSH terms: Huntingtin Protein, Animals, Brain, Exocytosis, Humans, Huntington Disease, Mutation, Nerve Tissue Proteins, Synaptic Transmission, Nuclear Proteins, Synapses, Transcription, Genetic, Models, Animal

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Synaptic dysfunction in Huntington's disease: a new perspective

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