Results 31 to 40 of about 93,886 (309)

Imbalance of p75(NTR)/TrkB protein expression in Huntington's disease: Implication for neuroprotective therapies [PDF]

, 2013
Neuroprotective therapies based on brain-derived neurotrophic factor (BDNF) administration have been proposed for Huntington's disease (HD) treatment. However, our group has recently reported reduced levels of TrkB in HD mouse models and HD human brain ...
Alberch, J., Brito, V., Del Toro Ruiz, D., Gines, S., Giralt, A., Puigdellivol, M.   +5 more
core   +1 more source

Oxidative stress parameters in plasma of Huntington's disease patients, asymptomatic Huntington's disease gene carriers and healthy subjects : a cross-sectional study [PDF]

, 2007
BACKGROUND : Animal data and postmortem studies suggest a role of oxidative stress in the Huntington's disease (HD), but in vivo human studies have been scarce.
Babić, Tomislav, Hećimović, Silva, Klepac, Nataša, Klepac, Ratimir, Relja, Maja, Trkulja, Vladimir   +5 more
core   +1 more source

Comprehensive behavioral testing in the R6/2 mouse model of Huntington's disease shows no benefit from CoQ10 or minocycline [PDF]

, 2010
Previous studies of the effects of coenzyme Q10 and minocycline on mouse models of Huntington’s disease have produced conflicting results regarding their efficacy in behavioral tests.
Allan J. Tobin, Benjamin Zahasky, Daniela Brunner, David Howland, Ethan Signer, Janet Leeds, Jennie Minnich, Jennifer Goodman, Larry Park, Liliana B. Menalled, Monica Patry, Natalie Ragland, Phillip A. S. Lowden, Xiao-Jiang Li   +13 more
core   +9 more sources

Intrabodies Binding the Proline-Rich Domains of Mutant Huntingtin Increase Its Turnover and Reduce Neurotoxicity [PDF]

, 2008
Although expanded polyglutamine (polyQ) repeats are inherently toxic, causing at least nine neurodegenerative diseases, the protein context determines which neurons are affected.
Bugg, Charles W., Dunn, Denise E., Khoshnan, Ali, Lo, Donald C., Patterson, Paul H., Southwell, Amber L.   +5 more
core   +2 more sources

The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington’s disease patients

Scientific Reports, 2017
We have previously shown that exon 1 of the huntingtin gene does not always splice to exon 2 resulting in the production of a small polyadenylated mRNA (HTTexon1) that encodes the highly pathogenic exon 1 HTT protein.
Andreas Neueder, Christian Landles, Rhia Ghosh, David Howland, Richard H. Myers, Richard L. M. Faull, Sarah J. Tabrizi, Gillian P. Bates   +7 more
doaj   +1 more source

Case report: Cerebellar sparing in juvenile Huntington's disease

Frontiers in Neurology, 2023
Juvenile Huntington's disease is an early-onset variant of Huntington's disease, generally associated with large CAG repeats and distinct clinical symptoms. The role of the cerebellum in Huntington's disease has been reevaluated, based on the presence of
Bruno Lopes Santos-Lobato, Bruno Lopes Santos-Lobato, Jéssica Santos de Souza Rocha, Luciano Chaves Rocha   +3 more
doaj   +1 more source

The Huntington's disease mutation impairs Huntingtin's role in the transport of NF-κB from the synapse to the nucleus [PDF]

, 2010
Expansion of a polyglutamine (polyQ) tract in the Huntingtin (Htt) protein causes Huntington's disease (HD), a fatal inherited neurodegenerative disorder.
Baquet, Bates, Bennett, Bhakar, Blahos, Caviston, Chen, Cho, Colin, Colomer, Dragatsis, Dunkley, Edoardo Marcora, Engelender, Fagerlund, Fridmacher, Gauthier, Graham, Gray, Gutekunst, Hanz, Harkiolaki, Hayden, Hering, Hickey, Hunt, Imarisio, Jordan, Joshi, Kaltschmidt, Kaltschmidt, Kaltschmidt, Kennedy, Korkin, Kornau, Leavitt, Leavitt, Li, Li, Li, Lipsky, Ma, Marcora, Marini, Marini, Mary B. Kennedy, Mattson, Meffert, Meffert, Menalled, Mikenberg, Mosammaparast, Otis, Patterson, Perlson, Shimojo, Simmons, Song, Strand, Sun, Suzuki, Takano, The Huntington's Disease Collaborative Research Group, Thompson, Tian, Towbin, Trushina, Velier, Wang, Wellmann, Xie, Zhang, Zuccato, Zuccato, Zuccato   +74 more
core   +4 more sources

Basal ganglia role in learning rewarded actions and executing previously learned choices: Healthy and diseased states [PDF]

, 2020
The basal ganglia (BG) is a collection of nuclei located deep beneath the cerebral cortex that is involved in learning and selection of rewarded actions. Here, we analyzed BG mechanisms that enable these functions.
Atwood, Brady, Kuznetsov, Alexey, Mulcahy, Garrett   +2 more
core   +1 more source

Laser Evoked Potentials in Early and Presymptomatic Huntington’s Disease

Behavioural Neurology, 2016
Pain was rarely studied in Huntington’s disease (HD). We presently aimed to extend our previous study on pain pathways functions by laser evoked potentials (LEPs) to a larger cohort of early unmedicated HD patients and a small group of presymptomatic HD (
Marina de Tommaso, Giovanni Franco, Katia Ricci, Anna Montemurno, Vittorio Sciruicchio   +4 more
doaj   +1 more source

Another Perspective on Huntington’s Disease: Disease Burden in Family Members and Pre-Manifest HD When Compared to Genotype-Negative Participants from ENROLL-HD

Brain Sciences, 2021
Background: In addition to the effects on patients suffering from motor-manifest Huntington’s disease (HD), this fatal disease is devasting to people who are at risk, premanifest mutation-carriers, and especially to whole families. There is a huge burden
Jannis Achenbach, Carsten Saft
doaj   +1 more source