Experimental models of HD and reflection on therapeutic strategies

J Kim, OL Bordiuk, RJ Ferrante - International review of neurobiology, 2011 - Elsevier
Huntington's disease (HD) is an autosomal dominant, progressive, and fatal
neurodegenerative disorder caused by an expanded polyglutamine cytosine–adenine–
guanine repeat in the gene coding for the protein huntingtin. Despite great progress over the
past two decades since the identification of the gene mutation, a direct causative pathway
from the HD gene mutation to neuronal dysfunction and death has not yet been established.
One important advance in understanding the pathogenic mechanisms of this disease has …