Huntington disease

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

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

Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease

Ravikumar, B., Vacher, C., Berger, Z., Davies, J. E., Luo, S., Oroz, L. G., Scaravilli, F., Easton, D. F., Duden, R., O'Kane, C. J., et al. (2004) · Nature Genetics · doi:10.1038/ng1362 · Cited by 2,347 · see citing works

A polymorphic DNA marker genetically linked to Huntington's disease

Gusella, J. F., Wexler, N. S., Conneally, P. M., Naylor, S. L., Anderson, M. A., Tanzi, R. E., Watkins, P. C., Ottina, K. A., Wallace, M. R., Sakaguchi, A. Y., et al. (1983) · Nature · doi:10.1038/306234a0 · Cited by 2,340 · see citing works

Unified Huntington's disease rating scale: Reliability and consistency

Kremer, H. P. H. (1996) · Movement Disorders · doi:10.1002/mds.870110204 · Cited by 2,109 · see citing works

Huntington's disease: from molecular pathogenesis to clinical treatment

Ross, C. A., & Tabrizi, S. J. (2010) · The Lancet Neurology · doi:10.1016/s1474-4422(10)70245-3 · Cited by 1,751 · see citing works

Therapeutic genome editing: prospects and challenges

Cox, D. B. T., Platt, R. J., & Zhang, F. (2015) · Nature Medicine · doi:10.1038/nm.3793 · Cited by 1,336 · see citing works

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

Beal, M. F., Kowall, N. W., Ellison, D. W., Mazurek, M. F., Swartz, K. J., & Martin, J. B. (1986) · Nature · doi:10.1038/321168a0 · Cited by 1,287 · 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

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

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

Huntington's disease: a clinical review

Roos, R. A. C. (2010) · Orphanet Journal of Rare Diseases · doi:10.1186/1750-1172-5-40 · Cited by 1,049 · see citing works

Treatment of infantile-onset spinal muscular atrophy with nusinersen: a phase 2, open-label, dose-escalation study

Finkel, R. S., Chiriboga, C. A., Vajsar, J., Day, J., Montes, J., Vivo, D. C. D., Yamashita, M., Rigo, F., Hung, G., Schneider, E., et al. (2016) · The Lancet · doi:10.1016/s0140-6736(16)31408-8 · Cited by 1,019 · see citing works

Huntington disease: natural history, biomarkers and prospects for therapeutics

Ross, C. A., Aylward, E. H., Wild, E. J., Langbehn, D. R., Long, J. D., Warner, J. H., Scahill, R. I., Leavitt, B. R., Stout, J. C., Paulsen, J. S., et al. (2014) · Nature Reviews Neurology · doi:10.1038/nrneurol.2014.24 · Cited by 1,016 · see citing works

Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data

Tabrizi, S. J., Langbehn, D. R., Leavitt, B. R., Roos, R. A., Durr, A., Craufurd, D., Kennard, C., Hicks, S. L., Fox, N. C., Scahill, R. I., et al. (2009) · The Lancet Neurology · doi:10.1016/s1474-4422(09)70170-x · Cited by 984 · see citing works

Cargo recognition failure is responsible for inefficient autophagy in Huntington’s disease

Martinez-Vicente, M., Talloczy, Z., Wong, E., Tang, G., Koga, H., Kaushik, S., de Vries, R., Arias, E., Harris, S., Sulzer, D., et al. (2010) · Nature Neuroscience · doi:10.1038/nn.2528 · Cited by 885 · see citing works

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

Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data

Tabrizi, S. J., Scahill, R. I., Owen, G. N., Dürr, A., Leavitt, B. R., Roos, R. A. C., Borowsky, B., Landwehrmeyer, G. B., Frost, C. D., Johnson, H. J., et al. (2013) · The Lancet Neurology · doi:10.1016/s1474-4422(13)70088-7 · Cited by 834 · see citing works

A new model for prediction of the age of onset and penetrance for Huntington's disease based on CAG length

Langbehn, D. R., Brinkman, R. R., Falush, D., Paulsen, J. S., Hayden, M. R., & on behalf of an International Huntington's Disease Collaborative Group (2004) · Clinical Genetics · doi:10.1111/j.1399-0004.2004.00241.x · Cited by 820 · 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

Detection of Huntington's disease decades before diagnosis: the Predict-HD study

Paulsen, J. S., Langbehn, D. R., Stout, J. C., Aylward, E., Ross, C. A., NANCE, M. A., Guttman, M., Johnson, S., MacDonald, M. E., Beglinger, L. J., et al. (2007) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2007.128728 · Cited by 808 · see citing works

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

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

Sustained Therapeutic Reversal of Huntington's Disease by Transient Repression of Huntingtin Synthesis

Kordasiewicz, H. B., Stanek, L. M., Wancewicz, E. V., Mazur, C., McAlonis, M. M., Pytel, K. A., Artates, J. W., Weiss, A., Cheng, S. H., Shihabuddin, L. S., et al. (2012) · Neuron · doi:10.1016/j.neuron.2012.05.009 · Cited by 770 · see citing works

Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset

The U.S.–Venezuela Collaborative Research Project*, & Wexler, N. S. (2004) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0308679101 · Cited by 761 · see citing works

Huntington disease: Clinical care and evaluation

Shoulson, I., & Fahn, S. (1979) · Neurology · doi:10.1212/wnl.29.1.1 · Cited by 746 · see citing works

Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington's disease

Snell, R. G., MacMillan, J., Cheadle, J. P., Fenton, I., Lazarou, L., Davies, P. J., MacDonald, M. E., Gusella, J. F., Harper, P. S., & Shaw, D. J. (1993) · Nature Genetics · doi:10.1038/ng0893-393 · Cited by 710 · see citing works

RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model

Harper, S. Q., Staber, P. D., He, X., Eliason, S. L., Martins, I. H., Mao, Q., Yang, L., Kotin, R. M., Paulson, H. L., & Davidson, B. L. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0501507102 · Cited by 682 · see citing works

A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease

Björkqvist, M., Wild, E. J., Thiele, J., Silvestroni, A., André, R., Lahiri, N., Raibon, E., Lee, R. V., Benn, C., Soulet, D., et al. (2008) · The Journal of Experimental Medicine · doi:10.1084/jem.20080178 · Cited by 646 · see citing works

Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

Ona, V. O., Li, M., Vonsattel, J. P., Andrews, L. J., Khan, S. Q., Chung, W. M., Frey, A. S., Menon, A., Li, X., Stieg, P. E., et al. (1999) · Nature · doi:10.1038/20446 · Cited by 640 · see citing works

An antisense oligonucleotide against SOD1 delivered intrathecally for patients with SOD1 familial amyotrophic lateral sclerosis: a phase 1, randomised, first-in-man study

Miller, T. M., Pestronk, A., David, W., Rothstein, J., Simpson, E., Appel, S. H., Andres, P. L., Mahoney, K., Allred, P., Alexander, K., et al. (2013) · The Lancet Neurology · doi:10.1016/s1474-4422(13)70061-9 · Cited by 617 · see citing works

Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis

Tabrizi, S. J., Scahill, R. I., Dürr, A., Roos, R. A. C., Leavitt, B. R., Jones, R., Landwehrmeyer, G. B., Fox, N. C., Johnson, H. J., Hicks, S. L., et al. (2010) · The Lancet Neurology · doi:10.1016/s1474-4422(10)70276-3 · Cited by 597 · see citing works

Astrocyte Kir4.1 ion channel deficits contribute to neuronal dysfunction in Huntington's disease model mice

Tong, X., Ao, Y., Faas, G. C., Nwaobi, S. E., Xu, J., Haustein, M. D., Anderson, M. A., Mody, I., Olsen, M. L., Sofroniew, M. V., et al. (2014) · Nature Neuroscience · doi:10.1038/nn.3691 · Cited by 592 · see citing works

Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data

Tabrizi, S. J., Reilmann, R., Roos, R. A. C., Dürr, A., Leavitt, B. R., Owen, G. N., Jones, R., Johnson, H. J., Craufurd, D., Hicks, S. L., et al. (2011) · The Lancet Neurology · doi:10.1016/s1474-4422(11)70263-0 · Cited by 540 · see citing works

Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease

Sathasivam, K., Neueder, A., Gipson, T. A., Landles, C., Benjamin, A. C., Bondulich, M. K., Smith, D. L., Faull, R. L. M., Roos, R. A. C., Howland, D., et al. (2013) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1221891110 · Cited by 532 · see citing works

Phenotypic characterization of individuals with 30-40 CAG repeats in the Huntington disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36-39 repeats.

Rubinsztein, D. C., Leggo, J., Coles, R., Almqvist, E., Biancalana, V., Cassiman, J. J., Chotai, K. A., Connarty, M., Crauford, D., Curtis, A., et al. (1996) · PubMed Central · Cited by 525 · see citing works

Kynurenine 3-Monooxygenase Inhibition in Blood Ameliorates Neurodegeneration

Zwilling, D., Huang, S., Sathyasaikumar, K. V., Notarangelo, F. M., Guidetti, P., Wu, H., Lee, J. H., Truong, J. K., Andrews-Zwilling, Y. S., Hsieh, E., et al. (2011) · Cell · doi:10.1016/j.cell.2011.05.020 · Cited by 519 · see citing works

Global changes to the ubiquitin system in Huntington's disease

Bennett, E. J., Shaler, T. A., Woodman, B., Ryu, K.-Y., Zaitseva, T. S., Becker, C. H., Bates, G. P., Schulman, H., & Kopito, R. R. (2007) · Nature · doi:10.1038/nature06022 · Cited by 497 · see citing works

Neuropsychiatric aspects of Huntington’s disease.

Paulsen, J. S. (2001) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp.71.3.310 · Cited by 486 · see citing works

Tetrabenazine as antichorea therapy in Huntington disease: A randomized controlled trial

Huntington Study Group (2006) · Neurology · doi:10.1212/01.wnl.0000198586.85250.13 · Cited by 471 · see citing works

Microglial activation correlates with severity in Huntington disease: A clinical and PET study

Pavese, N., Gerhard, A., Tai, Y. F., Ho, A. K., Turkheimer, F., Barker, R. A., Brooks, D. J., & Piccini, P. (2006) · Neurology · doi:10.1212/01.wnl.0000222734.56412.17 · Cited by 462 · see citing works

Motor and cognitive improvements in patients with Huntington's disease after neural transplantation

Bachoud‐Lévi, A., Rémy, P., Nguyen, J., Brugières, P., Lefaucheur, J., Bourdet, C., Baudic, S., Gaura-Schmidt, V., Maison, P., Haddad, B., et al. (2000) · The Lancet · doi:10.1016/s0140-6736(00)03310-9 · Cited by 461 · see citing works

Microglial activation in presymptomatic Huntington's disease gene carriers

Tai, Y. F., Pavese, N., Gerhard, A., Tabrizi, S. J., Barker, R. A., Brooks, D. J., & Piccini, P. (2007) · Brain · doi:10.1093/brain/awm044 · Cited by 440 · see citing works

Effect of Deutetrabenazine on Chorea Among Patients With Huntington Disease

Huntington Study Group, Frank, S., Testa, C. M., Stamler, D. A., Kayson, E., Davis, C. S., Edmondson, M. C., Kinel, S., Leavitt, B. R., Oakes, D., et al. (2016) · JAMA · doi:10.1001/jama.2016.8655 · Cited by 417 · see citing works

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

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

Neurocognitive signs in prodromal Huntington disease.

Stout, J. C., The PREDICT-HD Investigators and Coordinators of the Huntington Study Group, Paulsen, J. S., Queller, S., Solomon, A. C., Whitlock, K. B., Campbell, J. C., Carlozzi, N. E., Duff, K., Beglinger, L. J., et al. (2010) · Neuropsychology · doi:10.1037/a0020937 · Cited by 384 · see citing works

Dramatic tissue-specific mutation length increases are an early molecular event in Huntington disease pathogenesis

Kennedy, L. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg352 · Cited by 384 · see citing works

Protein aggregates in Huntington's disease

Arrasate, M., & Finkbeiner, S. M. (2011) · Experimental Neurology · doi:10.1016/j.expneurol.2011.12.013 · Cited by 381 · see citing works

CAG‐repeat length and the age of onset in Huntington disease (HD): A review and validation study of statistical approaches

Langbehn, D. R., Hayden, M. R., Paulsen, J. S., & and the PREDICT‐HD Investigators of the Huntington Study Group (2009) · American Journal of Medical Genetics Part B Neuropsychiatric Genetics · doi:10.1002/ajmg.b.30992 · Cited by 367 · see citing works

Placebo-Controlled Trial of Oral Laquinimod for Multiple Sclerosis

Comı, G., Jeffery, D., Kappos, L., Montalbán, X., Boyко, A., Rocca, M. A., & Filippi, M. (2012) · New England Journal of Medicine · doi:10.1056/nejmoa1104318 · Cited by 364 · see citing works

Neurofilament light protein in blood as a potential biomarker of neurodegeneration in Huntington's disease: a retrospective cohort analysis

Byrne, L. M., Rodrigues, F. B., Blennow, K., Durr, A., Leavitt, B. R., Roos, R. A. C., Scahill, R. I., Tabrizi, S. J., Zetterberg, H., Langbehn, D., et al. (2017) · The Lancet Neurology · doi:10.1016/s1474-4422(17)30124-2 · Cited by 364 · see citing works

Beyond the brain: widespread pathology in Huntington's disease

van der Burg, J. M. M., Björkqvist, M., & Brundin, P. (2009) · The Lancet Neurology · doi:10.1016/s1474-4422(09)70178-4 · Cited by 362 · see citing works

Effect of fetal neural transplants in patients with Huntington's disease 6 years after surgery: a long-term follow-up study

Bachoud‐Lévi, A., Gaura-Schmidt, V., Brugières, P., Lefaucheur, J., Boissé, M., Maison, P., Baudic, S., Ribeiro, M. J., Bourdet, C., Rémy, P., et al. (2006) · The Lancet Neurology · doi:10.1016/s1474-4422(06)70381-7 · Cited by 357 · see citing works

RAN Translation in Huntington Disease

Báñez-Coronel, M., Ayhan, F., Tarabochia, A. D., Zu, T., Pérez, B., Tusi, S. K., Pletniková, O., Borchelt, D., Ross, C. A., Margolis, R. L., et al. (2015) · Neuron · doi:10.1016/j.neuron.2015.10.038 · Cited by 356 · see citing works

Mutant Huntingtin promotes autonomous microglia activation via myeloid lineage-determining factors

Crotti, A., Benner, C., Kerman, B. E., Gosselin, D., Lagier-Tourenne, C., Zuccato, C., Cattaneo, E., Gage, F. H., Cleveland, D. W., & Glass, C. K. (2014) · Nature Neuroscience · doi:10.1038/nn.3668 · Cited by 333 · see citing works

Somatic expansion of the Huntington's disease CAG repeat in the brain is associated with an earlier age of disease onset

Swami, M., Hendricks, A. E., Gillis, T., Massood, T., Mysore, J. S., Myers, R. H., & Wheeler, V. C. (2009) · Human Molecular Genetics · doi:10.1093/hmg/ddp242 · Cited by 330 · see citing works

Proteolysis of Mutant Huntingtin Produces an Exon 1 Fragment That Accumulates as an Aggregated Protein in Neuronal Nuclei in Huntington Disease

Landles, C., Sathasivam, K., Weiss, A., Woodman, B., Moffitt, H., Finkbeiner, S. M., Sun, B., Gafni, J., Ellerby, L., Trottier, Y., et al. (2010) · Journal of Biological Chemistry · doi:10.1074/jbc.m109.075028 · Cited by 328 · see citing works

Psychopathology in Verified Huntington's Disease Gene Carriers

van Duijn, E., Kingma, E. M., & van der Mast, R. C. (2007) · Journal of Neuropsychiatry · doi:10.1176/jnp.2007.19.4.441 · Cited by 323 · see citing works

Guidelines for the molecular genetics predictive test in Huntington's disease

(1994) · Neurology · doi:10.1212/wnl.44.8.1533 · Cited by 310 · see citing works

Potent and Selective Antisense Oligonucleotides Targeting Single-Nucleotide Polymorphisms in the Huntington Disease Gene / Allele-Specific Silencing of Mutant Huntingtin

Carroll, J. B., Warby, S. C., Southwell, A. L., Doty, C. N., Greenlee, S., Skotte, N. H., Hung, G., Bennett, C. F., Freier, S. M., & Hayden, M. R. (2011) · Molecular Therapy · doi:10.1038/mt.2011.201 · Cited by 294 · see citing works

Permanent inactivation of Huntington's disease mutation by personalized allele-specific CRISPR/Cas9

Shin, J. W., Kim, K., Chao, M. J., Atwal, R. S., Gillis, T., MacDonald, M. E., Gusella, J. F., & Lee, J. (2016) · Human Molecular Genetics · doi:10.1093/hmg/ddw286 · Cited by 281 · see citing works

Up-regulation of GLT1 expression increases glutamate uptake and attenuates the Huntington's disease phenotype in the R6/2 mouse

Miller, B. R., Dorner, J. L., Shou, M., Sari, Y., Barton, S. J., Sengelaub, D. R., Kennedy, R. T., & Rebec, G. V. (2008) · Neuroscience · doi:10.1016/j.neuroscience.2008.02.004 · Cited by 280 · see citing works

Regional Specificity of Brain Atrophy in Huntington's Disease

Halliday, G. M., McRitchie, D. A., Macdonald, V., Double, K. L., Trent, R. J., & McCusker, E. A. (1998) · Experimental Neurology · doi:10.1006/exnr.1998.6919 · Cited by 271 · see citing works

Depression and Stages of Huntington's Disease

Paulsen, J. S., Nehl, C., Hoth, K. F., Kanz, J. E., Benjamin, M., Conybeare, R. A., McDowell, B. D., & Turner, B. M. (2005) · Journal of Neuropsychiatry · doi:10.1176/jnp.17.4.496 · Cited by 269 · see citing works

Quantification of mutant huntingtin protein in cerebrospinal fluid from Huntington’s disease patients

Wild, E. J., Boggio, R., Langbehn, D. R., Robertson, N., Salman, H., Miller, J. R. C., Zetterberg, H., Leavitt, B. R., Kuhn, R. R., Tabrizi, S. J., et al. (2015) · Journal of Clinical Investigation · doi:10.1172/jci80743 · Cited by 265 · see citing works

Five siRNAs Targeting Three SNPs May Provide Therapy for Three-Quarters of Huntington's Disease Patients

Pfister, E. L., Kennington, L., Straubhaar, J., Wagh, S., Liu, W., DiFiglia, M., Landwehrmeyer, B., Vonsattel, J.-P., Zamore, P. D., & Aronin, N. (2009) · Current Biology · doi:10.1016/j.cub.2009.03.030 · Cited by 261 · see citing works

Huntington's disease

Novak, M. J. U., & Tabrizi, S. J. (2010) · BMJ · doi:10.1136/bmj.c3109 · Cited by 255 · see citing works

Longitudinal change in regional brain volumes in prodromal Huntington disease

Aylward, E., Nopoulos, P., Ross, C. A., Langbehn, D. R., Pierson, R. K., Mills, J. A., Johnson, H. J., Magnotta, V. A., Juhl, A. R., Paulsen, J. S., et al. (2010) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2010.208264 · Cited by 253 · see citing works

Intergenerational and striatal CAG repeat instability in Huntington's disease knock-in mice involve different DNA repair genes

Dragileva, E., Hendricks, A. E., Teed, A. M., Gillis, T., Lopez, E., Friedberg, E. C., Kucherlapati, R., Edelmann, W., Lunetta, K. L., MacDonald, M. E., et al. (2008) · Neurobiology of Disease · doi:10.1016/j.nbd.2008.09.014 · Cited by 247 · see citing works

Preclinical Safety of RNAi-Mediated HTT Suppression in the Rhesus Macaque as a Potential Therapy for Huntington's Disease

McBride, J. L., Pitzer, M. R., Boudreau, R. L., Dufour, B., Hobbs, T., Ojeda, S. R., & Davidson, B. L. (2011) · Molecular Therapy · doi:10.1038/mt.2011.219 · Cited by 247 · see citing works

Bilateral human fetal striatal transplantation in Huntington’s disease

Hauser, R. A., Furtado, S., Cimino, C. R., Delgado, H., Eichler, S., Schwartz, S. E., Scott, D., Nauert, G. M., Soety, E. M., Sossi, V., et al. (2002) · Neurology · doi:10.1212/wnl.58.5.687 · Cited by 245 · see citing works

Treatment of Huntington's Disease

Frank, S. (2013) · Neurotherapeutics · doi:10.1007/s13311-013-0244-z · Cited by 232 · see citing works

RESEARCH ARTICLE: Systematic Assessment of BDNF and Its Receptor Levels in Human Cortices Affected by Huntington's Disease

Zuccato, C., Marullo, M., Conforti, P., MacDonald, M. E., Tartari, M., & Cattaneo, E. (2008) · Brain Pathology · doi:10.1111/j.1750-3639.2007.00111.x · Cited by 231 · see citing works

Critical Periods of Suicide Risk in Huntington’s Disease

Paulsen, J. S., Hoth, K. F., Nehl, C., Stierman, L., & The Huntington Study Group (2005) · American Journal of Psychiatry · doi:10.1176/appi.ajp.162.4.725 · Cited by 226 · see citing works

Neuropsychiatry of Huntington’s Disease and Other Basal Ganglia Disorders

Rosenblatt, A., & Leroi, I. (2000) · Psychosomatics · doi:10.1016/s0033-3182(00)71170-4 · Cited by 224 · see citing works

Safety of Converting From Tetrabenazine to Deutetrabenazine for the Treatment of Chorea

Frank, S., Stamler, D. A., Kayson, E., Claassen, D. O., Colcher, A., Davis, C. S., Duker, A. P., Eberly, S. W., Elmer, L. W., Furr-Stimming, E. E., et al. (2017) · JAMA Neurology · doi:10.1001/jamaneurol.2017.1352 · Cited by 213 · see citing works

Histone deacetylase inhibitors as therapeutics for polyglutamine disorders

Butler, R. K., & Bates, G. P. (2006) · Nature reviews. Neuroscience · doi:10.1038/nrn1989 · Cited by 212 · see citing works

Factors Associated With Slow Progression in Huntington's Disease

Myers, R. H., Sax, D. S., Koroshetz, W. J., Mastromauro, C. A., Cupples, L. A., Kiely, D. K., Pettengill, F. K., & Bird, E. D. (1991) · Archives of Neurology · doi:10.1001/archneur.1991.00530200036015 · Cited by 208 · see citing works

Prevalence of adult Huntington's disease in the UK based on diagnoses recorded in general practice records

Evans, S. J. W., Douglas, I., Rawlins, M. D., Wexler, N. S., Tabrizi, S. J., & Smeeth, L. L. (2013) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp-2012-304636 · Cited by 208 · see citing works

Characterization and localization of the Huntington disease gene product

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<i>C9orf72</i> expansions are the most common genetic cause of Huntington disease phenocopies

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