New avenues for the treatment of Huntington's disease

A Kim, K Lalonde, A Truesdell, P Gomes Welter… - International journal of …, 2021 - mdpi.com
Huntington's disease (HD) is a neurodegenerative disorder caused by a CAG expansion in
the HD gene. The disease is characterized by neurodegeneration, particularly in the striatum …

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 …

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 …

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 …

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 …

Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin

RK Graham, Y Deng, EJ Slow, B Haigh, N Bissada… - Cell, 2006 - cell.com
Cleavage of huntingtin (htt) has been characterized in vitro, and accumulation of caspase
cleavage fragments represents an early pathological change in brains of Huntington's …

Altered microRNA regulation in Huntington's disease models

ST Lee, K Chu, WS Im, HJ Yoon, JY Im, JE Park… - Experimental …, 2011 - Elsevier
Huntington's disease (HD) is a genetic neurodegenerative disease caused by abnormal
CAG expansion. MicroRNAs (miRNAs) are short RNA molecules regulating gene …

Palmitoylation of huntingtin by HIP14is essential for its trafficking and function

A Yanai, K Huang, R Kang, RR Singaraja… - Nature …, 2006 - nature.com
Post-translational modification by the lipid palmitate is crucial for the correct targeting and
function of many proteins. Here we show that huntingtin (htt) is normally palmitoylated at …

Silencing mutant huntingtin by adeno-associated virus-mediated RNA interference ameliorates disease manifestations in the YAC128 mouse model of Huntington's …

LM Stanek, SP Sardi, B Mastis, AR Richards… - Human gene …, 2014 - liebertpub.com
Huntington's disease (HD) is a fatal autosomal dominant neurodegenerative disease
caused by an increase in the number of polyglutamine residues in the huntingtin (Htt) …

Huntington's disease: can mice lead the way to treatment?

ZR Crook, D Housman - Neuron, 2011 - cell.com
Mouse models for Huntington's Disease (HD) and HD patients demonstrate motor and
behavioral dysfunctions, such as progressive loss of coordination and memory, and share …