Molecular mechanisms and potential therapeutical targets in Huntington's disease
C Zuccato, M Valenza, E Cattaneo - Physiological reviews, 2010 - journals.physiology.org
Huntington's disease (HD) is a neurodegenerative disorder caused by a CAG repeat
expansion in the gene encoding for huntingtin protein. A lot has been learned about this …
expansion in the gene encoding for huntingtin protein. A lot has been learned about this …
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 …
animal models of this disorder have been developed. A frequently asked question is: which …
[HTML][HTML] CRISPR/Cas9-mediated gene editing ameliorates neurotoxicity in mouse model of Huntington's disease
Huntington's disease is a neurodegenerative disorder caused by a polyglutamine repeat in
the Huntingtin gene (HTT). Although suppressing the expression of mutant HTT (mHTT) has …
the Huntingtin gene (HTT). Although suppressing the expression of mutant HTT (mHTT) has …
Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175
LB Menalled, AE Kudwa, S Miller, J Fitzpatrick… - PloS one, 2012 - journals.plos.org
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder
characterized by motor, cognitive and psychiatric manifestations. Since the mutation …
characterized by motor, cognitive and psychiatric manifestations. Since the mutation …
[HTML][HTML] Transcriptional repression of PGC-1α by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration
L Cui, H Jeong, F Borovecki, CN Parkhurst, N Tanese… - Cell, 2006 - cell.com
Huntington's disease (HD) is an inherited neurodegenerative disease caused by a
glutamine repeat expansion in huntingtin protein. Transcriptional deregulation and altered …
glutamine repeat expansion in huntingtin protein. Transcriptional deregulation and altered …
A slipped-CAG DNA-binding small molecule induces trinucleotide-repeat contractions in vivo
M Nakamori, GB Panigrahi, S Lanni, T Gall-Duncan… - Nature …, 2020 - nature.com
In many repeat diseases, such as Huntington's disease (HD), ongoing repeat expansions in
affected tissues contribute to disease onset, progression and severity. Inducing contractions …
affected tissues contribute to disease onset, progression and severity. Inducing contractions …
Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease
EJ Slow, J van Raamsdonk, D Rogers… - Human molecular …, 2003 - academic.oup.com
An expanded CAG repeat is the underlying genetic defect in Huntington disease, a disorder
characterized by motor, psychiatric and cognitive deficits and striatal atrophy associated with …
characterized by motor, psychiatric and cognitive deficits and striatal atrophy associated with …
Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice
M Gray, DI Shirasaki, C Cepeda, VM André… - Journal of …, 2008 - Soc Neuroscience
To elucidate the pathogenic mechanisms in Huntington's disease (HD) elicited by
expression of full-length human mutant huntingtin (fl-mhtt), a bacterial artificial chromosome …
expression of full-length human mutant huntingtin (fl-mhtt), a bacterial artificial chromosome …
Pathophysiology of Huntington's disease: time-dependent alterations in synaptic and receptor function
LA Raymond, VM André, C Cepeda, CM Gladding… - Neuroscience, 2011 - Elsevier
Huntington's disease (HD) is a progressive, fatal neurological condition caused by an
expansion of CAG (glutamine) repeats in the coding region of the Huntington gene. To date …
expansion of CAG (glutamine) repeats in the coding region of the Huntington gene. To date …
ALOX5-mediated ferroptosis acts as a distinct cell death pathway upon oxidative stress in Huntington's disease
Although it is well established that Huntington's disease (HD) is mainly caused by
polyglutamine-expanded mutant huntingtin (mHTT), the molecular mechanism of mHTT …
polyglutamine-expanded mutant huntingtin (mHTT), the molecular mechanism of mHTT …