Article
Dominant-Negative Effects of Adult-Onset Huntingtin Mutations Alter the Division of Human Embryonic Stem Cells-Derived Neural Cells.
PloS one - 1 Jan 2016
Lopes Carla, Aubert Sophie, Bourgois-Rocha Fany, Barnat Monia, Rego Ana Cristina, Déglon Nicole, Perrier Anselme L, Humbert Sandrine
Abstract excerpt
Mutations of the huntingtin protein (HTT) gene underlie both adult-onset and juvenile forms of Huntington's disease (HD). HTT modulates mitotic spindle orientation and cell fate in mouse cortical progenitors from the ventricular zone. Using human embryonic stem cells (hESC) characterized as carrying mutations associated with adult-onset disease during pre-implantation genetic diagnosis, we investigated the...
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