Results 11 to 20 of about 52,129 (204)

Proteostasis in striatal cells and selective neurodegeneration in Huntington’s disease [PDF]

Frontiers in Cellular Neuroscience, 2014
Selective neuronal loss is a hallmark of neurodegenerative diseases, including Huntington’s disease (HD). Although mutant huntingtin, the protein responsible for Huntington’s disease, is expressed ubiquitously, a subpopulation of neurons in the striatum ...
Julia eMargulis, Julia eMargulis, Steven eFinkbeiner, Steven eFinkbeiner, Steven eFinkbeiner   +4 more
doaj   +6 more sources

The Ubiquitin-Proteasome System in Huntington’s Disease: Are Proteasomes Impaired, Initiators of Disease, or Coming to the Rescue? [PDF]

Biochemistry Research International, 2012
Huntington’s disease is a progressive neurodegenerative disease, caused by a polyglutamine expansion in the huntingtin protein. A prominent hallmark of the disease is the presence of intracellular aggregates initiated by N-terminal huntingtin fragments ...
Sabine Schipper-Krom, Katrin Juenemann, Eric A. J. Reits   +2 more
doaj   +2 more sources

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   +7 more sources

Clinical and genetic analysis of 29 Brazilian patients with Huntington’s disease-like phenotype [PDF]

, 2011
Huntington’s disease (HD) is a neurodegenerative disorder characterized by chorea, behavioral disturbances and dementia, caused by a pathological expansion of the CAG trinucleotide in the HTT gene. Several patients have been recognized with the typical
Adrian Danek, Alexis Brice, Andrew SE, Bauer I, Bauer P, Bayreuther C, Benedikt Bader, Cellini E, Costa MC, Cécile Cazeneuve, Dobson-Stone C, Guilherme Riccioppo Rodrigues, Hirose G, Holmes SE, IBGE, Iscia Lopes-Cendes, Keckarević M, Krause A, Kremer B, Margolis RL, Martins S, McNeill A, Odile Russaouen, Rodrigues GR, Rodrigues GR, Rodriguez-Revenga L, Rosenblatt A, Ruth H. Walker, Santos C, Schmitz-Hübsch T, Stevanin G, Sułek-Piatkowska A, Teive HAG, Vitor Tumas, Vuillaume I, Wardle M, Warner JP, Wild EJ, Wilson Marques Jr.   +38 more
core   +3 more sources

Nutritional management of individuals with Huntington’s disease: nutritional guidelines [PDF]

, 2012
The delivery of good nutritional care is a fundamental element of the management of individuals with Huntington’s disease and all patients with Huntington’s disease will, at some time, need dietary intervention because of the sequela of the disease; yet ...
Brotherton, Ailsa M, Campos, Lillian, Rae, Daniela, Rowell, Arleen, Simpson, Sheila A, Zoia, Vanessa   +5 more
core   +1 more source

MODELLING GAIT SYNDROME IN HUNTINGTON’S DISEASE: THE GENETIC ALGORITHM APPROACH

Nigerian Journal of Technology, 2017
Huntington’s disease (HD) which usually affects the patients at middle age results from malfunctioning of the basal ganglia. It is characterized by cognitive impairment, involuntary movements, neuropsychiatric and psychological disturbances. Early motor signs of Huntington’s disease typically include the gradual onset of clumsiness, balance ...
Ooe Ajibola, L Ogunwolu
openaire   +4 more sources

Gene suppression approaches to neurodegeneration

Alzheimer’s Research & Therapy, 2017
Gene suppression approaches have emerged over the last 20 years as a novel therapeutic approach for the treatment of neurodegenerative diseases. These include RNA interference and anti-sense oligonucleotides, both of which act at the post-transcriptional
Rhia Ghosh, Sarah J. Tabrizi
doaj   +1 more source

Silencing Srsf6 does not modulate incomplete splicing of the huntingtin gene in Huntington’s disease models

Scientific Reports, 2020
We have previously shown that the incomplete splicing of exon 1 to exon 2 of the HTT gene results in the production of a small polyadenylated transcript (Httexon1) that encodes the highly pathogenic exon 1 HTT protein.
Michael A. Mason, Casandra Gomez-Paredes, Kirupa Sathasivam, Andreas Neueder, Aikaterini-Smaragdi Papadopoulou, Gillian P. Bates   +5 more
doaj   +1 more source

A fruitful fly forward : the role of the fly in drug discovery for neurodegeneration [PDF]

, 2014
AD, Alzheimer’s disease; APP, amyloid precursor protein; BBB, blood brain barrier; GFP, green fluorescent protein; HTS, high-throughput screening; HD, Huntington’s disease; LB, Lewy bodies; PD, Parkinson’s disease; PolyQ, Polyglutamine; RNAi, RNA ...
Briffa, Michelle, Cauchi, Ruben J., Vassallo, Neville   +2 more
core   +1 more source

Ubiquitin–proteasome system involvement in Huntington’s disease [PDF]

Frontiers in Molecular Neuroscience, 2014
Huntington's disease (HD) is a genetic autosomal dominant neurodegenerative disease caused by the expansion of a CAG repeat in the huntingtin (htt) gene. This triplet expansion encodes a polyglutamine stretch (polyQ) in the N-terminus of the high molecular weight (348-kDa) and ubiquitously expressed protein htt. Normal individuals have between 6 and 35
Ortega, Zaira, Lucas, José Javier
openaire   +4 more sources