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217 Newest Publications about the topic huntington's disease
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Selective histone deacetylase (HDAC) inhibition imparts beneficial effects in Huntington's disease mice: implications for the ubiquitin–proteasomal and autophagy systems
15-12-2012 | Haiqun Jia; Ryan J. Kast; Joan S. Steffan; Elizabeth A. Thomas, Human Molecular Genetics, 2012
We previously demonstrated that the histone deacetylase (HDAC) inhibitor, 4b, which preferentially targets HDAC1 and HDAC3, ameliorates Huntington's disease (HD)-related phenotypes in different HD model systems. In the current study, we investigated extensive behavioral and biological ...
The effects of multidisciplinary rehabilitation in patients with early‐to‐middle‐stage Huntington's disease: a pilot study
08-12-2012 | J. A. Thompson, T. M. Cruickshank, L. E. Penailillo, J. W. Lee, R. U. Newton, R. A. Barker, M. R. Ziman, European Journal of Neurology, 2012
Background and purpose Despite advances in the understanding of Huntington's disease (HD), treatment remains symptomatic. Multidisciplinary rehabilitation, however, appears to impact disease progression. Here we show the feasibility, safety and efficacy of a 9‐month multidisciplinary ...
A Genome-Scale RNA–Interference Screen Identifies RRAS Signaling as a Pathologic Feature of Huntington's Disease
29-11-2012 | John P. Miller et al., PLoS Genetics, 2012
by John P. Miller, Bridget E. Yates, Ismael Al-Ramahi, Ari E. Berman, Mario Sanhueza, Eugene Kim, Maria de Haro, Francesco DeGiacomo, Cameron Torcassi, Jennifer Holcomb, Juliette Gafni, Sean D. Mooney, Juan Botas, Lisa M. Ellerby, Robert E. Hughes A genome-scale RNAi screen was ...
Downregulation of cannabinoid receptor 1 from neuropeptide Y interneurons in the basal ganglia of patients with Huntington's disease and mouse models
21-11-2012 | Eric A. Horne, Jonathan Coy, Katie Swinney, Susan Fung, Allison E. T. Cherry, William R. Marrs, Alipi V. Naydenov, Y ..., European Journal of Neuroscience, 2012
Abstract Cannabinoid receptor 1 (CB1 receptor) controls several neuronal functions, including neurotransmitter release, synaptic plasticity, gene expression and neuronal viability. Downregulation of CB1 expression in the basal ganglia of patients with Huntington's disease (HD) and animal ...
Neil1 is a genetic modifier of somatic and germline CAG trinucleotide repeat instability in R6/1 mice
15-11-2012 | Linda Møllersen; Alexander D. Rowe; Jennifer L. Illuzzi; Gunn A. Hildrestrand; Katharina J. Gerhold; Linda Tveterås; ..., Human Molecular Genetics, 2012
Huntington's disease (HD) is a progressive neurodegenerative disorder caused by trinucleotide repeat (TNR) expansions. We show here that somatic TNR expansions are significantly reduced in several organs of R6/1 mice lacking exon 2 of Nei-like 1 (Neil1) (R6/1/Neil1–/–), when compared with ...
Evaluation of exclusion prenatal and exclusion preimplantation genetic diagnosis for Huntington's disease in the Netherlands
08-11-2012 | M.C. Rij, C.E.M. Die‐Smulders, E.K. Bijlsma, G.M.W.R. Wert, J.P. Geraedts, R.A.C. Roos, A. Tibben, Clinical Genetics, 2012
Abstract Background Individuals at 50% risk of Huntington's disease (HD) who prefer not to know their carrier status, might opt for exclusion prenatal diagnosis (ePND) or exclusion preimplantation genetic diagnosis (ePGD). This study aims to provide a better understanding of couples’ ...
Distinct Neurogenomic States in Basal Ganglia Subregions Relate Differently to Singing Behavior in Songbirds
08-11-2012 | Austin T. Hilliard et al., PLoS Computational Biology, 2012
by Austin T. Hilliard, Julie E. Miller, Steve Horvath, Stephanie A. White Both avian and mammalian basal ganglia are involved in voluntary motor control. In birds, such movements include hopping, perching and flying. Two organizational features that distinguish the songbird basal ...
Synthetic zinc finger repressors reduce mutant huntingtin expression in the brain of R6/2 mice [Neuroscience]
06-11-2012 | Mireia Garriga-Canut; Carmen Agustín-Pavón; Frank Herrmann; Aurora Sánchez; Mara Dierssen; Cristina Fillat; Mark Isalan, Proceedings of the National Academy of Sciences current issue, 2012
Huntington's disease (HD) is a dominantly inherited neurodegenerative disorder caused by expanded CAG repeats in the huntingtin (HTT) gene. Although several palliative treatments are available, there is currently no cure and patients generally die 10–15 y after diagnosis. Several promising ...
Pharmacokinetics of cysteamine bitartrate following intraduodenal delivery
01-11-2012 | Ranjan Dohil, Betty L. Cabrera, Jon A. Gangoiti, Bruce A. Barshop, Patrice Rioux, Fundamental & Clinical Pharmacology, 2012
Abstract Cysteamine is approved for the treatment of cystinosis and is being evaluated for Huntington's disease and non‐alcoholic fatty liver disease. Little is known about the bioavailability and biodistribution of the drug. The aim was to determine plasma, cerebrospinal fluid (CSF), and ...
O-GlcNAc cycling modulates neurodegeneration [Commentary]
23-10-2012 | Cheng-Xin Gong; Fei Liu; Khalid Iqbal, Proceedings of the National Academy of Sciences current issue, 2012
Age-associated neurodegenerative diseases are not only a great scientific challenge but a medical, economic, and social burden of enormous dimensions. This family of diseases includes Alzheimer’s (AD), Parkinson disease, Huntington disease, and others, among which AD is the most common. AD ...
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