Results 21 to 30 of about 4,891,805 (263)

Proteostasis in striatal cells and selective neurodegeneration in Huntington’s disease

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   +1 more source

Mutant huntingtin fragmentation in immune cells tracks Huntington's disease progression. [PDF]

, 2012
Huntington's disease (HD) is a fatal, inherited neurodegenerative disorder caused by an expanded CAG repeat in the gene encoding huntingtin (HTT). Therapeutic approaches to lower mutant HTT (mHTT) levels are expected to proceed to human trials, but ...
Salman Haider, Weiss, A, Rachael I. Scahill, Sarah J. Tabrizi, Ulrike Träger, Bates, G, Stephan Grueninger, Wild, Edward J., Weiss, Andreas, Scahill, R, Ralph Andre, Farmer, Ruth, Macdonald, Douglas, Haider, Salman, Macdonald, D, Scahil, Rachael I., Grueninger, Stephan, Chris Frost, Frost, C, Farmer, R, Wild, E, Bilbe, G, Tabrizi, SJ, Ruth Farmer, Tabrizi, Sarah J., Landles, C, Nayana Lahiri, Gillian P. Bates, Edward J. Wild, Scahill, Rachael I, Douglas Macdonald, Rainer Kuhn, Andre, R, Frost, Chris, Kuhn, R, Grueninger, S, Bates, GP, Wild, Edward J, Wild, EJ, Kuhn, Rainer, Graeme Bilbe, Träger, U, Andreas Weiss, Christian Landles, Lahiri, N, Tabrizi, Sarah J, Scahill, RI, Andre, Ralph, Landles, Christian, Traeger, Ulrike, Lahiri, Nayana, Träger, Ulrike, Bilbe, Graeme, Haider, S, Bates, Gillian P, Bates, Gillian P.   +55 more
core   +1 more source

Juvenile Huntington’s disease: two case reports and a review of the literature

Journal of Medical Case Reports, 2020
Background Huntington’s disease is a rare, autosomal dominant neurodegenerative disease characterized by motor, cognitive, and psychiatric symptoms. Usually, the disease symptoms first appear around the age of 40, but in 5–10% of cases, they manifest ...
Sigita Lesinskienė, Darja Rojaka, Rūta Praninskienė, Aušra Morkūnienė, Aušra Matulevičienė, Algirdas Utkus   +5 more
doaj   +1 more source

Cholinergic deficits selectively boost cortical intratelencephalic control of striatum in male Huntington’s disease model mice

Nature Communications, 2023
The corticostriatal dysfunction underlying Huntington’s disease remains incompletely understood. Here, the authors find increased intratelencephalic connectivity resulting from deficient cholinergic transmission in a mouse model of Huntington’s disease.
Tristano Pancani, Michelle Day, Tatiana Tkatch, David L. Wokosin, Patricia González-Rodríguez, Jyothisri Kondapalli, Zhong Xie, Yu Chen, Vahri Beaumont, D. James Surmeier   +9 more
doaj   +1 more source

Monkey hybrid stem cells develop cellular features of Huntington's disease

BMC Cell Biology, 2010
Background Pluripotent stem cells that are capable of differentiating into different cell types and develop robust hallmark cellular features are useful tools for clarifying the impact of developmental events on neurodegenerative diseases such as ...
Lorthongpanich Chanchao, Johnson Zach, Yang Shang-Hsun, Snyder Brooke R, Cheng Pei-Hsun, Cheng Eric CH, Laowtammathron Chuti, Kuo Hung-Chih, Parnpai Rangsun, Chan Anthony WS   +9 more
doaj   +1 more source

Visual system integrity and cognition in early Huntington's disease [PDF]

, 2014
Posterior cortical volume changes and abnormal visuomotor performance are present in patients with Huntington's disease (HD). However, it is unclear whether posterior cortical volume loss contributes to abnormal neural activity, and whether activity ...
Landwehrmeyer, G Bernhard, Ratheiser I., Süssmuth, Sigurd D., Orth, Michael, Depping, Malte S, Nenad Vasic, Wolf, Robert C, Ratheiser, Iris, Iris Ratheiser, Vasic N., Sigurd D. Süssmuth, Depping M. S., Thomann P. A., Thomann, Philipp A., Malte S. Depping, Bernhard Landwehrmeyer, Georg, Süssmuth, Sigurd S, Georg Bernhard Landwehrmeyer, Sprengelmeyer R., Sprengelmeyer, Reiner, Fabio Sambataro, Vasic, Nenad, Sambataro, Fabio, Thomann, Philipp A, Orth M., Depping, Malte S., Philipp A. Thomann, Eva‐Maria Baldas, Reiner Sprengelmeyer, Wolf R. C., Sprengelmeyer, Reiner Heinrich, Robert C. Wolf, Bernhard Landwehrmeyer G., Baldas, Eva Maria, Michael Orth, Baldas E. -M., Sambataro F., Baldas, Eva-Maria, Sussmuth S. D.   +38 more
core   +1 more source

Increased central microglial activation associated with peripheral cytokine levels in premanifest Huntington's disease gene carriers

Neurobiology of Disease, 2015
Previous studies have shown activation of the immune system and altered immune response in Huntington's disease (HD) gene carriers. Here, we hypothesized that peripheral and central immune responses could be concurrent pathophysiological events and ...
Marios Politis, Nayana Lahiri, Flavia Niccolini, Paul Su, Kit Wu, Paolo Giannetti, Rachael I. Scahill, Federico E. Turkheimer, Sarah J. Tabrizi, Paola Piccini   +9 more
doaj   +1 more source

Postoperative hematoma in cervical spondylosis patient complicated with Huntington’s disease: Case report and literature review

SAGE Open Medical Case Reports, 2023
Hematoma is a life-threatening complication of anterior surgery in cervical spondylosis patients. Herein, we report a cervical spondylosis patient complicated with Huntington’s disease, who developed unexpected neck hematoma after anterior cervical ...
Zifan Zhang, Chen Xu, Guoqing Wen, Minjie Dong, Xiaolong Shen, Baofeng Gong, Baifeng Sun, Min Qi, Ye Tian, Yang Liu, Wen Yuan   +10 more
doaj   +1 more source

Huntington's Disease

Pediatric Neurology Briefs, 1989
The positron-emission tomography (PET) findings in a seven year old girl with the juvenile form of Huntington's disease are described from the Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, Montreal, Canada.
J Gordon Millichap
doaj   +1 more source

Visual control improves the accuracy of hand positioning in Huntington’s disease

Aktualności Neurologiczne, 2017
Background: The study aimed at demonstrating dependence of visual feedback during hand and finger positioning task performance among Huntington’s disease patients in comparison to patients with Parkinson’s disease and cervical dystonia.
Emilia J. Sitek, Witold Sołtan, Agnieszka Konkel, Dariusz Wieczorek, Michał Schinwelski, Piotr Robowski, Jarosław Sławek   +6 more
doaj   +1 more source