Results 71 to 80 of about 10,552,439 (323)

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

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

Additional file 7: of The dynamics of early-state transcriptional changes and aggregate formation in a Huntingtonâ s disease cell model

, 2017
Expression profiles for all fourteen clusters created using the CLICK algorithm (Expander). Expression patterns for all clusters were standardized to mean zero and standard deviation one. Error bars correspond to one standard deviation. (PDF 360 kb)
Hagen, Martijn Van, Piebes, Diewertje, Leeuw, Wim De, Vuist, Ilona, Roon-Mom, Willeke Van, Moerland, Perry, Pernette Verschure   +6 more
openaire   +1 more source

Identification and evaluation of biomarkers for Huntington’s disease [PDF]

, 2009
Huntington’s disease (HD) is a devastating, incurable inherited neurodegenerative disorder that commonly affects adults in mid-life. Despite encouraging results from in vitro and animal trials, disease-modifying therapeutic trials in HD are limited by ...
Wild, E.J.
core  

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

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

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

Faecal microbiota transplant ameliorates gut dysbiosis and cognitive deficits in Huntington’s disease mice

Brain Communications, 2022
Huntington’s disease is a neurodegenerative disorder involving psychiatric, cognitive and motor symptoms. Huntington’s disease is caused by a tandem-repeat expansion in the huntingtin gene, which is widely expressed throughout the brain and body ...
C. Gubert, J. Choo, Chloe J Love, S. Kodikara, B. Masson, J. Liew, Yiwen Wang, Geraldine Kong, V. Narayana, T. Renoir, Kim-Anh Lê Cao, G. Rogers, A. Hannan   +12 more
semanticscholar   +1 more source

A transgenic minipig models of Huntington’s disease [PDF]

, 2013
Background: Some promising treatments for Huntington’s disease (HD) may require pre-clinical testing in large animals. Minipig is a suitable species because of its large gyrencephalic brain and long lifespan. Objective: To generate HD transgenic (TgHD)
P. Vodicka, J. Klima, J. Motlik, M. Baxa, M. Marsala, P. Musilova, S. Juhas, R. Vrtel, J. Schier, M. Hruska Plochan, H. Hansikova, S. Kubickova, J. Juhasova, A. Weiss, D. S. Howland, M. DiFiglia, T. Nejime, A. Pavlok, M. Macakova, A. Miyanohara, E. M. Sontag, L. M. Thompson, E. Cattaneo, S. Marsala   +23 more
core   +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