Results 51 to 60 of about 37,609 (272)

Factor analysis of behavioural symptoms in Huntington's disease

, 2011
A principal-components factor analysis was performed on behavioural data obtained from the European Huntington's Disease Network REGISTRY study. 1690 valid assessments using the United Huntington's Disease Rating Scale Behavioural Rating Scale were ...
ROMANO, SILVIA, Simpson, Sheila A., De Souza, Jenny, Van Walsem, Marleen, Landwehrmeyer, Bernhard, Van Duijn, Erik, European Huntington's Disease Network, Rickards, Hugh, SQUITIERI, Ferdinando   +8 more
core   +1 more source

Functional and Structural Evidence of Neurofluid Circuit Aberrations in Huntington Disease

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Disrupted neurofluid regulation may contribute to neurodegeneration in Huntington disease (HD). Because neurofluid pathways influence waste clearance, inflammation, and the distribution of central nervous system (CNS)–delivered therapeutics, understanding their dysfunction is increasingly important as targeted treatments emerge.
Kilian Hett, Abigail Dubois, Melanie Leguizamon, Alexander Song, Paula Trujillo, Colin D. McKnight, Ciaran M. Considine, Manus J. Donahue, Daniel O. Claassen   +8 more
wiley   +1 more source

Clinical impairment in premanifest and early Huntington's disease is associated with regionally specific atrophy.

, 2011
TRACK-HD is a multicentre longitudinal observational study investigating the use of clinical assessments and 3-Tesla magnetic resonance imaging as potential biomarkers for future therapeutic trials in Huntington's disease (HD).
Stout, J.C., Roos, Raymund AC, TRACK-HD investigators, Sarah J. Tabrizi, Langbehn, DR, David Craufurd, Langbehn, Douglas R, Callaghan, Jenny, Blair R. Leavitt, Alexandra Durr, Reilmann, R, Frajman, Ellen, Say, Miranda J, Henley, Susie M D, Johnson, H., Scahill, R.I., Roos, R.A.C., Rebecca Jones, Stephen L. Hicks, Craufurd, David, Keenan, Siobhan, Whitlock, Kathryn, Susie M.D. Henley, Fox, Nick, Queller, Sarah, Sturrock, Aaron, Say, M.J., Hobbs, N.Z., Milchman, Cassie, t'Hart, Ellen, Craufurd, D, Tabrizi, S.J., Bechtel, N., null null, Owen, Gail, Tabrizi, SJ, Leavitt, B.R., Miranda J. Say, Coleman, Allison, Henley, Susie MD, Jones, R., Jauffret, Céline, Hobbs, NZ, Landwehmeyer, Bernhard, Hyare, H., Jones, Rebecca, Wang, Chiachi, Natalie Bechtel, Dar Santos, Rachelle, Bechtel, N, Rosas, H Diana, Douglas R. Langbehn, Raymund A.C. Roos, Lehericy, S., Durr, A., Hoffman, Arndt, Bechtel, Natalie, Hicks, S.L., Christopher Kennard, Julie C. Stout, Stout, JC, Kennard, C, Henley, SM, Pepple, Tracey, Campbell, Colin, Langbehn, D.R., Jones, R, Rachael I. Scahill, Arran, Natalie, Reilmann, R., Harpreet Hyare, Lee, Stephanie, Henley, S.M.D., Durr, Alexandra, Ralf Reilmann, Campbell, Melissa, Mills, Jim, Axelson, Eric, Monaco, William, Hobbs, Nicola Z, Nicola Z. Hobbs, Bardinet, Eric, Say, MJ, Hicks, SL, Durr, A, Johnson, H, van Bogaard, Simon J A, Scahill, Rachael I, Lehéricy, S, Stout, Julie C, Leavitt, Blair R, Hyare, H, Valabre, Romain, Read, Joy, Ordidge, Roger, Hyare, Harpreet, Craufurd, D., Lehéricy, Stéphane, Decolongon, Joji, Stéphane Lehéricy, Roos, RA, Roos, Raymund A C, van der Grond, Jeroen, Kennard, C., Tabrizi, Sarah J, O'Regan, Alison, Scahill, RI, Marelli, Cecilia, Dumas, Eve, Lahiri, Nayana, Kennard, Christopher, Leavitt, BR, Hans Johnson, Reilmann, Ralf, Justo, Damian, Nigaud, Kevin, Johnson, Hans, Hicks, Stephen L   +117 more
core   +1 more source

Unraveling 4‐Phenylbutyrate's Therapeutic Role in SLC6A1 Disorders: Pharmacochaperoning Over HDAC Inhibition

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Variants in SLC6A1, encoding the GABA transporter 1 (GAT‐1), cause epilepsy, autism spectrum disorder, and developmental delay via loss of GABA uptake, impaired trafficking, and ER retention. We previously found that 4‐Phenylbutyrate (PBA), an FDA‐approved drug, restores GABA uptake and reduces seizures in SLC6A1‐related disorders ...
Melissa B. DeLeeuw, Karishma Randhave, Ekta Anand, Ziang (Debbie) Song, Wangzhen Shen, Jing‐Qiong Kang   +5 more
wiley   +1 more source

A Novel Class of “Super‐Strained” Spiro Heterocycles: Gateway to 1‐Azaspiro[3.3]heptane Derivatives, and Biological Validation

Angewandte Chemie, EarlyView.
A new class of “super‐strained” spiro heterocycles—spirocyclic 1‐azabicyclo[1.1.0]butanes—was synthesized via insertion of cyclobutane‐, oxetane‐, and azetidine‐containing sulfonium reagents into substituted azirines. The stability of this new class of compounds was studied.
Philipp Natho, Annarita Vicenti, Marco Colella, Francesco Pasca, Giuseppe Romanazzi, Mauro Niso, Carmen Abate, Ernesto Mesto, Emanuela Schingaro, Renzo Luisi   +9 more
wiley   +2 more sources

The Role of Innovation Technology in the Rehabilitation of Patients Affected by Huntington’s Disease: A Scoping Review

Biomedicines, 2023
Huntington’s disease is an autosomal dominant neurodegenerative disease caused by the repetition of cytosine, adenine, and guanine trinucleotides on the short arm of chromosome 4p16.3 within the Huntingtin gene.
Maria Grazia Maggio, Luana Billeri, Davide Cardile, Angelo Quartarone, Rocco Salvatore Calabrò   +4 more
doaj   +1 more source

Developing a Functional Polyelectrolyte Hybrid Layer Scaffold Coating and Evaluation of Its Performance for Interaction With Neural Cells

Advanced Materials Interfaces, EarlyView.
Polyelectrolyte scaffold coatings modified with Cu and Fe3O4 nanoparticles regulate neural stem cell behavior in vitro. Increased Fe3O4 content enhances mitochondrial activity and neuronal differentiation, whereas higher Cu levels reduce cell viability.
Anna Grzeczkowicz, Agata Lipko, Angelika Kwiatkowska, Marcin Strawski, Monika Drabik, Ludomira H. Granicka   +5 more
wiley   +1 more source

Targeting the cholinergic system to develop a novel therapy for Huntington's disease

, 2016
In this review, we outline the role of the cholinergic system in Huntington's disease, and briefly describe the dysfunction of cholinergic transmission, cholinergic neurons, cholinergic receptors and cholinergic survival factors observed in post-mortem ...
D'Souza, GX, Waldvogel, Henry
core   +1 more source

Avoidance as a strategy of (not) coping: qualitative interviews with carers of Huntington's Disease patients [PDF]

, 2005
Peer ...
Lowit, Alison, van Teijlingen Edwin R, Alison Lowit, van Teijlingen, Edwin R., Edwin R van Teijlingen, Lowit Alison, Van Teijlingen, Edwin R   +6 more
core   +1 more source

Engineering Microbial Particles for Next‐Generation Biomedical Platforms

Advanced Science, EarlyView.
Microbe‐derived particles (MDPs), which include extracellular vesicles, outer membrane vesicles, inclusion bodies, polysaccharide particles, and virus‐like particles, represent a rapidly expanding category of bioinspired nanomaterials. With their natural origin, intrinsic biocompatibility, and highly programmable functionality, MDPs serve as a ...
Yuting Li, Yunjie Lu, Ziyan Huang, Jianjiang Chen, Yanbin Liu, Lei Qin, Chao Wang, Yumin Wu   +7 more
wiley   +1 more source