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 …
neurodegenerative disorders. Among these, Huntington disease (HD), which is inherited in …
Brain-derived neurotrophic factor restores synaptic plasticity in a knock-in mouse model of Huntington's disease
G Lynch, EA Kramar, CS Rex, Y Jia… - Journal of …, 2007 - Soc Neuroscience
Asymptomatic Huntington's disease (HD) patients exhibit memory and cognition deficits that
generally worsen with age. Similarly, long-term potentiation (LTP), a form of synaptic …
generally worsen with age. Similarly, long-term potentiation (LTP), a form of synaptic …
The selective vulnerability of nerve cells in Huntington's disease
KA Sieradzan, DMA Mann - Neuropathology and applied …, 2001 - Wiley Online Library
It is now more than 7 years since the genetic mutation causing Huntington's disease (HD)
was first identified. Unstable CAG expansion in the IT15 gene, responsible for disease, is …
was first identified. Unstable CAG expansion in the IT15 gene, responsible for disease, is …
TRiC subunits enhance BDNF axonal transport and rescue striatal atrophy in Huntington's disease
Corticostriatal atrophy is a cardinal manifestation of Huntington's disease (HD). However,
the mechanism (s) by which mutant huntingtin (mHTT) protein contributes to the …
the mechanism (s) by which mutant huntingtin (mHTT) protein contributes to the …
Knock-in mouse models of Huntington's disease
LB Menalled - NeuroRx, 2005 - Springer
Huntington's disease is an autosomal dominant neurodegenerative disorder that is
characterized by motor, cognitive, and psychiatric alterations. The mutation responsible for …
characterized by motor, cognitive, and psychiatric alterations. The mutation responsible for …
Differential ubiquitination and degradation of huntingtin fragments modulated by ubiquitin-protein ligase E3A
KP Bhat, S Yan, CE Wang, S Li… - Proceedings of the …, 2014 - National Acad Sciences
Ubiquitination of misfolded proteins, a common feature of many neurodegenerative
diseases, is mediated by different lysine (K) residues in ubiquitin and alters the levels of …
diseases, is mediated by different lysine (K) residues in ubiquitin and alters the levels of …
The HdhQ150/Q150 knock-in mouse model of HD and the R6/2 exon 1 model develop comparable and widespread molecular phenotypes
The identification of the Huntington's disease (HD) mutation as a CAG/polyglutamine repeat
expansion enabled the generation of transgenic rodent models and gene-targeted mouse …
expansion enabled the generation of transgenic rodent models and gene-targeted mouse …
Of mice, rats and men: revisiting the quinolinic acid hypothesis of Huntington's disease
R Schwarcz, P Guidetti, KV Sathyasaikumar… - Progress in …, 2010 - Elsevier
The neurodegenerative disease Huntington's disease (HD) is caused by an expanded
polyglutamine (polyQ) tract in the protein huntingtin (htt). Although the gene encoding htt …
polyglutamine (polyQ) tract in the protein huntingtin (htt). Although the gene encoding htt …
Caenorhabditis elegans as a model system for target identification and drug screening against neurodegenerative diseases
L Ma, Y Zhao, Y Chen, B Cheng, A Peng… - European Journal of …, 2018 - Elsevier
Over the past decades, Caenorhabditis elegans (C. elegans) has been widely used as a
model system because of its small size, transparent body, short generation time and lifespan …
model system because of its small size, transparent body, short generation time and lifespan …
Mouse models of Huntington's disease
PP Farshim, GP Bates - Huntington's disease, 2018 - Springer
The identification of the mutation causing Huntington's disease (HD) has led to the
generation of a large number of mouse models. These models are used to further enhance …
generation of a large number of mouse models. These models are used to further enhance …