Cortical thickness measured from MRI in the YAC128 mouse model of Huntington's disease

JP Lerch, JB Carroll, A Dorr, S Spring, AC Evans… - Neuroimage, 2008 - Elsevier
A recent study found differences in localised regions of the cortex between the YAC128
mouse model of Huntington's Disease (HD) and wild-type mice. There are, however, few …

Cholesterol biosynthesis pathway is disturbed in YAC128 mice and is modulated by huntingtin mutation

M Valenza, JB Carroll, V Leoni… - Human molecular …, 2007 - academic.oup.com
Our recent analyses of the cholesterol biosynthetic pathway in Huntington's disease (HD)
cells, in the R6/2 huntingtin-fragment mouse model of HD as well as in human tissues have …

Phosphorylation of huntingtin reduces the accumulation of its nuclear fragments

SC Warby, CN Doty, RK Graham, J Shively… - Molecular and Cellular …, 2009 - Elsevier
Huntingtin is phosphorylated on serine-421 (S421) by the pro-survival signaling protein
kinases Akt and SGK. Phosphorylation of huntingtin at S421 is variable in different regions of …

Palmitoylation and function of glial glutamate transporter-1 is reduced in the YAC128 mouse model of Huntington disease

K Huang, MH Kang, C Askew, R Kang… - Neurobiology of …, 2010 - Elsevier
Excitotoxicity plays a key role in the selective vulnerability of striatal neurons in Huntington
disease (HD). Decreased glutamate uptake by glial cells could account for the excess …

Genetic mouse models of Huntington's disease: focus on electrophysiological mechanisms

C Cepeda, DM Cummings, VM André… - ASN …, 2010 - journals.sagepub.com
The discovery of the HD (Huntington's disease) gene in 1993 led to the creation of genetic
mouse models of the disease and opened the doors for mechanistic studies. In particular …

Testicular degeneration in Huntington disease

JM Van Raamsdonk, Z Murphy, DM Selva… - Neurobiology of …, 2007 - Elsevier
Huntington disease (HD) is an adult onset, neurodegenerative disorder that results from
CAG expansion in the HD gene. Recent work has demonstrated testicular degeneration in …

[HTML][HTML] A CAG repeat-targeting artificial miRNA lowers the mutant huntingtin level in the YAC128 model of Huntington's disease

A Kotowska-Zimmer, L Przybyl, M Pewinska… - … Therapy-Nucleic Acids, 2022 - cell.com
Among the many proposed therapeutic strategies for Huntington's disease (HD), allele-
selective therapies are the most desirable but also the most challenging. RNA interference …

Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain

JM Van Raamsdonk, M Metzler, E Slow, J Pearson… - Neurobiology of …, 2007 - Elsevier
The YAC128 mouse model of Huntington disease (HD) exhibits motor abnormalities,
cognitive dysfunction and selective neuropathology which are similar to the human disease …

[HTML][HTML] Corticostriatal dysfunction in Huntington's disease: the basics

KD Bunner, GV Rebec - Frontiers in human neuroscience, 2016 - frontiersin.org
The main input to the basal ganglia, the corticostriatal pathway, shows some of the earliest
signs of neuropathology in Huntington's disease (HD), an inherited neurodegenerative …

[HTML][HTML] Characterization of behavioral, neuropathological, brain metabolic and key molecular changes in zQ175 knock-in mouse model of Huntington's disease

Q Peng, B Wu, M Jiang, J Jin, Z Hou, J Zheng, J Zhang… - PloS one, 2016 - journals.plos.org
Huntington's disease (HD) is caused by an expansion of the trinucleotide poly (CAG) tract
located in exon 1 of the huntingtin (Htt) gene leading to progressive neurodegeneration in …