Results 81 to 90 of about 44,176 (282)

Reduced Fractalkine Levels Lead to Striatal Synaptic Plasticity Deficits in Huntington’s Disease

Frontiers in Cellular Neuroscience, 2020
Huntington’s disease (HD) is an inherited neurodegenerative disorder in which the striatum is the most affected brain region. Although a chronic inflammatory microglial reaction that amplifies disease progression has been described in HD patients, some ...
Anya Kim, Anya Kim, Anya Kim, Anya Kim, Esther García-García, Esther García-García, Esther García-García, Esther García-García, Marco Straccia, Marco Straccia, Marco Straccia, Marco Straccia, Marco Straccia, Andrea Comella-Bolla, Andrea Comella-Bolla, Andrea Comella-Bolla, Andrea Comella-Bolla, Andrea Comella-Bolla, Andrés Miguez, Andrés Miguez, Andrés Miguez, Andrés Miguez, Andrés Miguez, Mercè Masana, Mercè Masana, Mercè Masana, Mercè Masana, Jordi Alberch, Jordi Alberch, Jordi Alberch, Jordi Alberch, Jordi Alberch, Josep M. Canals, Josep M. Canals, Josep M. Canals, Josep M. Canals, Josep M. Canals, Manuel J. Rodríguez, Manuel J. Rodríguez, Manuel J. Rodríguez, Manuel J. Rodríguez   +40 more
doaj   +1 more source

Sphingomyelin and GM1 Influence Huntingtin Binding to, Disruption of, and Aggregation on Lipid Membranes [PDF]

, 2018
Huntington disease (HD) is an inherited neurodegenerative disease caused by the expansion beyond a critical threshold of a polyglutamine (polyQ) tract near the N-terminus of the huntingtin (htt) protein. Expanded polyQ promotes the formation of a variety
Campbell, Warren A., Chaibva, Maxmore, Frey, Shelli L., Gao, Xiang, Jain, Pranav, Legleiter, Justin   +5 more
core   +4 more sources

Mitochondria and the Actin Cytoskeleton in Neurodegeneration

Cytoskeleton, EarlyView.
ABSTRACT Mitochondrial dysfunction and cytoskeletal disorganization are widely recognized hallmarks of neurodegenerative diseases such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), and amyotrophic lateral sclerosis (ALS).
Shivani Tuli, Preet Patel, Aneri Shethji, David Gau   +3 more
wiley   +1 more source

Huntingtin ( HTT ) [PDF]

Science-Business eXchange, 2012
  +10 more sources

Effects of High‐ and Low‐Fat Meals on the Bioavailability and Pharmacokinetics of Votoplam, a HTT Gene Splicing Modifier

Clinical Pharmacology in Drug Development, EarlyView.
Abstract Votoplam is a novel, orally bioavailable, small molecule HTT gene splicing modifier that is being developed for the treatment of Huntington's disease. This was a single dose, open‐label, two‐period, crossover food effect study that evaluated the effect of high‐ and low‐fat meals on 20 mg votoplam in healthy participants. There was a washout of
Lucy Lee, Amy‐Lee Richards, Nageswara Reddy, Brian Beers, Lee Golden, Ronald Kong   +5 more
wiley   +1 more source

Presynaptic APP levels and synaptic homeostasis are regulated by Akt phosphorylation of huntingtin

eLife, 2020
Studies have suggested that amyloid precursor protein (APP) regulates synaptic homeostasis, but the evidence has not been consistent. In particular, signaling pathways controlling APP transport to the synapse in axons and dendrites remain to be ...
Julie Bruyère, Yah-Se Abada, Hélène Vitet, Gaëlle Fontaine, Jean-Christophe Deloulme, Aurélia Cès, Eric Denarier, Karin Pernet-Gallay, Annie Andrieux, Sandrine Humbert, Marie-Claude Potier, Benoît Delatour, Frédéric Saudou   +12 more
doaj   +1 more source

MeCP2: a novel Huntingtin interactor [PDF]

Human Molecular Genetics, 2013
Transcriptional dysregulation has been proposed to play a major role in the pathology of Huntington's disease (HD). However, the mechanisms that cause selective downregulation of target genes remain unknown. Previous studies have shown that mutant huntingtin (Htt) protein interacts with a number of transcription factors thereby altering transcription ...
McFarland KN, Huizenga MN, Darnell SB, Sangrey GR, Berezovska O, Cha J-HJ, Outeiro TF, Sadri-Vakili G   +7 more
openaire   +4 more sources

Using a Brief Body Sway Assessment Device to Track Balance Differences across the Huntington's Disease Integrated Staging System Spectrum

Movement Disorders Clinical Practice, EarlyView.
Abstract Background Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by a mutation in the huntingtin gene on chromosome 4, leading to progressive cognitive decline, motor impairment, and functional disability. Although balance impairment is recognized in HD, its onset and evolution with disease stage remain poorly ...
Japleen Kaur, Nadeen Youhanan, Krisha Bagga, Andrew Hall, Paul E. Gilbert, Daniel J. Goble, Jody Corey‐Bloom   +6 more
wiley   +1 more source

Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models

Neurobiology of Disease, 2006
Huntington disease (HD) is a devastating neuropsychiatric disease caused by expansion of a trinucleotide repeat (CAG) in the HD gene. Neuropathological changes include the appearance of N-terminal huntingtin fragments, decreased brain weight and ...
Rona K. Graham, Elizabeth J. Slow, Yu Deng, Nagat Bissada, Ge Lu, Jacqueline Pearson, Jacqueline Shehadeh, Blair R. Leavitt, Lynn A. Raymond, Michael R. Hayden   +9 more
doaj   +1 more source

Huntington's Disease and Huntington's Disease‐like 2 (HDL2) in Martinique

Movement Disorders Clinical Practice, EarlyView.
ABSTRACT Background Huntington's Disease‐like 2 (HDL2), caused by a CAG repeat expansion in JPH3, closely resembles HD. All reported HDL2 patients to date have some African ancestry. While both disorders exist in the Caribbean, their relative frequency and clinical characteristics remain largely unknown.
Ignacio Antolin‐Sanfeliz, Anna‐Gaelle Giguet‐Valard, Sophie Duclos, Cécile Cazeneuve, Chloé Angelini, Aïssatou Signaté, Russell L. Margolis, Cyril Goizet, Rémi Bellance   +8 more
wiley   +1 more source