Results 11 to 20 of about 10,466,444 (219)

Cortical and Striatal Circuits in Huntington's Disease

Frontiers in Neuroscience, 2020
Huntington's disease (HD) is a hereditary neurodegenerative disorder that typically manifests in midlife with motor, cognitive, and/or psychiatric symptoms.
Blumenstock, S., Dudanova, I.
core   +2 more sources

A biological classification of Huntington's disease: the Integrated Staging System.

Lancet Neurology, 2022
The current research paradigm for Huntington's disease is based on participants with overt clinical phenotypes and does not address its pathophysiology nor the biomarker changes that can precede by decades the functional decline.
S. Tabrizi, S. Schobel, Emily C. Gantman, A. Mansbach, B. Borowsky, P. Konstantinova, T. Mestre, Jennifer Panagoulias, C. Ross, M. Zauderer, Ariana P. Mullin, Klaus Romero, Sudhir Sivakumaran, Emily C. Turner, J. Long, C. Sampaio   +15 more
semanticscholar   +1 more source

Potential disease modifying therapies for Huntington’s disease, lessons learned and future opportunities

Lancet Neurology, 2022
Huntington’s disease is the most frequent autosomal dominant neurodegenerative disorder, for which we have no approved disease-modifying treatments. The molecular pathogenesis of Huntington’s disease is complex, with toxicity arising from full length ...
S. Tabrizi, C. Estévez-Fraga, W. V. van Roon-Mom, M. Flower, R. Scahill, E. Wild, I. Muñoz-Sanjuán, C. Sampaio, A. Rosser, B. Leavitt   +9 more
semanticscholar   +1 more source

New Avenues for the Treatment of Huntington’s Disease

International Journal of Molecular Sciences, 2021
Huntington’s disease (HD) is a neurodegenerative disorder caused by a CAG expansion in the HD gene. The disease is characterized by neurodegeneration, particularly in the striatum and cortex.
A. Kim, Kathryn Lalonde, Aaron Truesdell, Priscilla Gomes Welter, P. Brocardo, T. Rosenstock, J. Gil-Mohapel   +6 more
semanticscholar   +1 more source

Huntington’s disease: diagnosis and management

Practical Neurology, 2021
Huntington’s disease (HD) is an inherited neurodegenerative disease characterised by neuropsychiatric symptoms, a movement disorder (most commonly choreiform) and progressive cognitive impairment. The diagnosis is usually confirmed through identification
T. Stoker, S. Mason, J. Greenland, S. Holden, Helen Santini, R. Barker   +5 more
semanticscholar   +1 more source

Abnormal molecular signatures of inflammation, energy metabolism, and vesicle biology in human Huntington disease peripheral tissues

Genome Biology, 2022
Background A major challenge in neurodegenerative diseases concerns identifying biological disease signatures that track with disease progression or respond to an intervention. Several clinical trials in Huntington disease (HD), an inherited, progressive
Andreas Neueder, Kerstin Kojer, Tanja Hering, Daniel J. Lavery, Jian Chen, Nathalie Birth, Jaqueline Hallitsch, Sonja Trautmann, Jennifer Parker, Michael Flower, Huma Sethi, Salman Haider, Jong-Min Lee, Sarah J. Tabrizi, Michael Orth   +14 more
doaj   +1 more source

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

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

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
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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