Article
Early transcriptional changes linked to naturally occurring Huntington's disease mutations in neural derivatives of human embryonic stem cells.
Human molecular genetics - 1 Sept 2012
Feyeux Maxime, Bourgois-Rocha Fany, Redfern Amanda, Giles Peter, Lefort Nathalie, Aubert Sophie, Bonnefond Caroline, Bugi Aurore, Ruiz Marta, Deglon Nicole, Jones Lesley, Peschanski Marc, Allen Nicholas D, Perrier Anselme L
Abstract excerpt
Huntington's disease (HD) is characterized by a late clinical onset despite ubiquitous expression of the mutant gene at all developmental stages. How mutant huntingtin impacts on signalling pathways in the pre-symptomatic period has remained essentially unexplored in humans due to a lack of appropriate models. Using multiple human embryonic stem cell lines derived from blastocysts diagnosed as carrying the mutant...
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