Light and electron microscopic characterization of the evolution of cellular pathology in YAC128 Huntington's disease transgenic mice

Z Bayram-Weston, L Jones, SB Dunnett… - Brain research bulletin, 2012 - Elsevier
Huntington's disease (HD) is a progressive neurodegenerative disease caused by the
insertion of an expanded polyglutamine sequence within the huntingtin protein. This …

Modulation of SETDB1 activity by APQ ameliorates heterochromatin condensation, motor function, and neuropathology in a Huntington's disease mouse model

YJ Hwang, SJ Hyeon, Y Kim, S Lim… - Journal of enzyme …, 2021 - Taylor & Francis
The present study describes evaluation of epigenetic regulation by a small molecule as the
therapeutic potential for treatment of Huntington's disease (HD). We identified 5-allyloxy-2 …

Protein misfolding detected early in pathogenesis of transgenic mouse model of Huntington disease using amyloid seeding assay

S Gupta, DW Colby - Journal of Biological Chemistry, 2012 - ASBMB
Huntington disease (HD) is one of several fatal neurodegenerative disorders associated
with misfolded proteins. Here, we report a novel method for the sensitive detection of …

Mouse models of Huntington's disease

SP Brooks, SB Dunnett - … of Huntington's Disease and Parkinson's Disease, 2015 - Springer
In this review, we explore the similarities and differences in the behavioural neurobiology
found in the mouse models of Huntington's disease (HD) and the human disease state. The …

Mouse models for validating preclinical candidates for Huntington's disease

XW Yang, M Gray - … of Huntington's Disease: Applications to Drug …, 2011 - books.google.com
Ever since its original description by George Huntington in 1872, Huntington's disease (HD)
has been known as one of the most devastating inherited neurodegenerative disorders …

CA150 expression delays striatal cell death in overexpression and knock-in conditions for mutant huntingtin neurotoxicity

M Arango, S Holbert, D Zala, E Brouillet… - Journal of …, 2006 - Soc Neuroscience
Transcriptional dysregulation caused by expanded polyglutamines (polyGlns) in huntingtin
(htt) may be central to cell-autonomous mechanisms for neuronal cell death in Huntington's …

Age‐dependent alterations of the kynurenine pathway in the YAC 128 mouse model of Huntington disease

G Mazarei, DP Budac, G Lu, H Adomat… - Journal of …, 2013 - Wiley Online Library
Abstract Indoleamine 2, 3 dioxygenase (Ido1), the first and rate‐limiting enzyme of the
kynurenine pathway (KP), is a striatally enriched gene with increased expression levels in …

Overview of Huntington's disease models: neuropathological, molecular, and behavioral differences

C Rangel‐Barajas, GV Rebec - Current protocols in …, 2018 - Wiley Online Library
Transgenic mouse models of Huntington's disease (HD), a neurodegenerative condition
caused by a single gene mutation, have been transformative in their ability to reveal the …

Compensatory changes in the ubiquitin–proteasome system, brain-derived neurotrophic factor and mitochondrial complex II/III in YAC72 and R6/2 transgenic mice …

H Seo, W Kim, O Isacson - Human molecular genetics, 2008 - academic.oup.com
Intraneuronal protein aggregates of the mutated huntingtin in Huntington's disease (HD)
brains suggest an overload and/or dysfunction of the ubiquitin–proteasome system (UPS) …

Neurobiology of Huntington's disease: applications to drug discovery

DC Lo, RE Hughes - 2010 - books.google.com
Highlighting advances in the discovery and development of new drug therapies for
neurodegenerative disorders, Neurobiology of Huntington's Disease focuses on the many …