A critical evaluation of adenosine A2A receptors as potentially “druggable” targets in Huntington's disease

P Popoli, D Blum, MR Domenici… - Current …, 2008 - ingentaconnect.com
Huntington's disease (HD) is a dominantly inherited neurodegenerative disorder caused by
the expansion of a polymorphic CAG trinucleotide repeat encoding a poly-glutamine tract …

Comparison of an expanded ataxia interactome with patient medical records reveals a relationship between macular degeneration and ataxia

JJ Kahle, N Gulbahce, CA Shaw, J Lim… - Human molecular …, 2011 - academic.oup.com
Abstract Spinocerebellar ataxias 6 and 7 (SCA6 and SCA7) are neurodegenerative
disorders caused by expansion of CAG repeats encoding polyglutamine (polyQ) tracts in …

A simple assay for direct visual and colorimetric sensing application of cysteamine using Au@ Ag core-shell nanoparticles

T Sasikumar, M Ilanchelian - Optical Materials, 2020 - Elsevier
The present study elucidates the preparation of colorimetric Au@ Ag core-shell
nanoparticles (Au@ Ag core-shell NPs) probe for sensitive, label-free and rapid sensing of …

Animal models for metabolic, neuromuscular and ophthalmological rare diseases

G Vaquer, FR Dannerstedt, M Mavris… - Nature Reviews Drug …, 2013 - nature.com
Animal models are important tools in the discovery and development of treatments for rare
diseases, particularly given the small populations of patients in which to evaluate …

Tonic mGluR5/CB1‐dependent suppression of inhibition as a pathophysiological hallmark in the striatum of mice carrying a mutant form of huntingtin

A Dvorzhak, M Semtner, DS Faber… - The Journal of …, 2013 - Wiley Online Library
Key points In neurodegenerative diseases, the afflicted brain provides both an important
object of study and an opportunity to characterize a given cellular interaction from a …

The group 2 metabotropic glutamate receptor agonist LY379268 rescues neuronal, neurochemical and motor abnormalities in R6/2 Huntington's disease mice

A Reiner, DC Lafferty, HB Wang, N Del Mar… - Neurobiology of …, 2012 - Elsevier
Excitotoxic injury to striatum by dysfunctional cortical input or aberrant glutamate uptake may
contribute to Huntington's disease (HD) pathogenesis. Since corticostriatal terminals …

NMDA receptor function and NMDA receptor-dependent phosphorylation of huntingtin is altered by the endocytic protein HIP1

M Metzler, L Gan, TP Wong, L Liu, J Helm… - Journal of …, 2007 - Soc Neuroscience
Huntingtin-interacting protein 1 (HIP1) is an endocytic adaptor protein that plays a role in
clathrin-mediated endocytosis and the ligand-induced internalization of AMPA receptors …

Mice lacking caspase-2 are protected from behavioral changes, but not pathology, in the YAC128 model of Huntington disease

JB Carroll, AL Southwell, RK Graham, JP Lerch… - Molecular …, 2011 - Springer
Abstract Background Huntington Disease (HD) is a neurodegenerative disorder in which
caspase activation and cleavage of substrates, including the huntingtin protein, has been …

[HTML][HTML] Increased body weight of the BAC HD transgenic mouse model of Huntington's disease accounts for some but not all of the observed HD-like motor deficits

AE Kudwa, LB Menalled, S Oakeshott, C Murphy… - PLoS …, 2013 - ncbi.nlm.nih.gov
The genome of the Bacterial Artificial Chromosome (BAC) transgenic mouse model of
Huntington's Disease (BAC HD) contains the 170 kb human HTT locus modified by the …

Hypothalamic and neuroendocrine changes in Huntington's disease

S Hult, K Schultz, R Soylu, A Petersen - Current drug targets, 2010 - ingentaconnect.com
Huntington's disease (HD) is neither a fatal hereditary neurodegenerative disorder without
satisfactory treatments nor a cure. It is caused by a CAG repeat expansion in the huntingtin …