Huntingtin and neurogenesis : from development to adulthood

Orateur : Sandrine HUMBERT (Institut Curie / CNRS UMR 3306 / INSERM U1005, Orsay)

The bulk of interest in the huntingtin protein has centered on the fact that, when mutated, huntingtin causes Huntington’s disease, a devastating neurodegenerative disorder. Given the adult onset and dysfunction and death of adult neurons characterizing Huntington’s disease, most studies have focused on the toxic effects elicited by mutant huntingtin in post-mitotic neurons. In contrast, the roles of the wild-type protein during development and adulthood have been overlooked. We will discuss how huntingtin regulates adult hippocampal neurogenesis and anxiety/depression-related behaviors in mice. Huntingtin is also important during development and affects division and cell fate of cortical progenitors of the ventricular zone in mouse embryos. We will show the consequences of the presence of an abnormal polyglutamine expansion in huntingtin during cortical neurogenesis and consider the viewing of HD as a developmental disorder.

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