Choosing an animal model for the study of Huntington's disease

MA Pouladi, AJ Morton, MR Hayden - Nature Reviews Neuroscience, 2013 - nature.com
Since the identification of the causative gene in Huntington's disease (HD), a number of
animal models of this disorder have been developed. A frequently asked question is: which …

Huntingtin and the Synapse

JC Barron, EP Hurley, MP Parsons - Frontiers in Cellular …, 2021 - frontiersin.org
Huntington disease (HD) is a monogenic disease that results in a combination of motor,
psychiatric and cognitive symptoms. HD is caused by a CAG trinucleotide repeat expansion …

The human motor cortex microcircuit: insights for neurodegenerative disease

P McColgan, J Joubert, SJ Tabrizi… - Nature Reviews …, 2020 - nature.com
The human motor cortex comprises a microcircuit of five interconnected layers with different
cell types. In this Review, we use a layer-specific and cell-specific approach to integrate …

Intrinsic mutant HTT-mediated defects in oligodendroglia cause myelination deficits and behavioral abnormalities in Huntington disease

C Ferrari Bardile, M Garcia-Miralles… - Proceedings of the …, 2019 - National Acad Sciences
White matter abnormalities are a nearly universal pathological feature of neurodegenerative
disorders including Huntington disease (HD). A long-held assumption is that this white …

An enhanced Q175 knock-in mouse model of Huntington disease with higher mutant huntingtin levels and accelerated disease phenotypes

AL Southwell, A Smith-Dijak, C Kay… - Human molecular …, 2016 - academic.oup.com
Huntington disease (HD) model mice with heterozygous knock-in (KI) of an expanded CAG
tract in exon 1 of the mouse huntingtin (Htt) gene homolog genetically recapitulate the …

Putting proteins in their place: palmitoylation in Huntington disease and other neuropsychiatric diseases

FB Young, SL Butland, SS Sanders, LM Sutton… - Progress in …, 2012 - Elsevier
Post-translational modification of proteins by the lipid palmitate is critical for protein
localization and function. Palmitoylation is regulated by the opposing enzymes palmitoyl …

Striatal synaptic dysfunction and altered calcium regulation in Huntington disease

LA Raymond - Biochemical and biophysical research communications, 2017 - Elsevier
Synaptic dysfunction and altered calcium homeostasis in the brain is common to many
neurodegenerative disorders. Among these, Huntington disease (HD), which is inherited in …

Structural and molecular myelination deficits occur prior to neuronal loss in the YAC128 and BACHD models of Huntington disease

RTY Teo, X Hong, L Yu-Taeger, Y Huang… - Human molecular …, 2016 - academic.oup.com
White matter (WM) atrophy is a significant feature of Huntington disease (HD), although its
aetiology and early pathological manifestations remain poorly defined. In this study, we …

[HTML][HTML] Anti-semaphorin 4D immunotherapy ameliorates neuropathology and some cognitive impairment in the YAC128 mouse model of Huntington disease

AL Southwell, S Franciosi, EB Villanueva, Y Xie… - Neurobiology of …, 2015 - Elsevier
Huntington disease (HD) is an inherited, fatal neurodegenerative disease with no disease-
modifying therapy currently available. In addition to characteristic motor deficits and atrophy …

FTY720 (fingolimod) is a neuroprotective and disease-modifying agent in cellular and mouse models of Huntington disease

A Di Pardo, E Amico, M Favellato… - Human molecular …, 2014 - academic.oup.com
Huntington disease (HD) is a genetic neurodegenerative disorder for which there is currently
no cure and no way to stop or even slow the brain changes it causes. In the present study …