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 …

[HTML][HTML] Mouse models of Huntington's disease and methodological considerations for therapeutic trials

RJ Ferrante - Biochimica et Biophysica Acta (BBA)-Molecular Basis …, 2009 - Elsevier
Huntington's disease (HD) is an autosomal dominant, progressive, and fatal
neurodegenerative disorder caused by an expanded polyglutamine cytosine–adenine …

Experimental therapeutics in transgenic mouse models of Huntington's disease

MF Beal, RJ Ferrante - Nature Reviews Neuroscience, 2004 - nature.com
Despite important advances in understanding and elucidating the molecular and
mechanistic pathways that mediate progression in Huntington's disease (HD), effective …

Transgenic animal models for study of the pathogenesis of Huntington's disease and therapy

R Chang, X Liu, S Li, XJ Li - Drug design, development and …, 2015 - Taylor & Francis
Huntington's disease (HD) is caused by a genetic mutation that results in polyglutamine
expansion in the N-terminal regions of huntingtin. As a result, this polyQ expansion leads to …

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 …

Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant …

A Kuhn, DR Goldstein, A Hodges… - Human molecular …, 2007 - academic.oup.com
To test the hypotheses that mutant huntingtin protein length and wild-type huntingtin dosage
have important effects on disease-related transcriptional dysfunction, we compared the …

Modeling Huntington's disease in cells, flies, and mice

S Sipione, E Cattaneo - Molecular neurobiology, 2001 - Springer
A milestone in Huntington's disease (HD) research is represented by the identification of the
causative gene. With the genetics at hand, a series of transgenic cellular and animal models …

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 …

Progressive cognitive deficit, motor impairment and striatal pathology in a transgenic Huntington disease monkey model from infancy to adulthood

AWS Chan, J Jiang, Y Chen, C Li, MS Prucha, Y Hu… - PloS one, 2015 - journals.plos.org
One of the roadblocks to developing effective therapeutics for Huntington disease (HD) is
the lack of animal models that develop progressive clinical traits comparable to those seen …

Mouse models of Huntington disease: variations on a theme

DE Ehrnhoefer, SL Butland… - Disease models & …, 2009 - journals.biologists.com
An accepted prerequisite for clinical trials of a compound in humans is the successful
alleviation of the disease in animal models. For some diseases, however, successful …