Start at 11:00AM
Speaker:
Sandrine Humbert
DR2 INSERM, team leader: « Neural progenitors and brain pathologies »
Grenoble Institut Neurosciences - INSERM U1216 - University Grenoble Alpes

Huntington Disease (HD) is a late-manifesting neurodegenerative disorder caused by an abnormal polyglutamine tract expansion in the huntingtin (HTT) protein. As with all polyglutamine diseases, symptoms typically do not appear until mid-life or later, although very long tracts can cause juvenile onset. Yet HTT and mutant HTT are expressed from the very beginning of life and HTT is essential for mouse development.

Anyway, given the adult onset and dysfunction and death of adult neurons characterizing HD, most studies have focused on the toxic effects elicited by mutant HTT in post-mitotic neurons and the roles of the wild-type protein during development have been overlooked.

We will discuss how HTT regulates several steps of mouse embryonic corticogenesis. HTT is crucial to maintain the pool of cycling progenitors and for the migration and post-natal maturation of post-mitotic neurons. We will describe the underlying molecular mechanisms by which HTT mediates its effects. Finally, we will also show the consequences of the presence of an abnormal polyglutamine expansion in HTT during cortical neurogenesis and consider the viewing of HD as a developmental disorder.

Seminar open to all interested researchers.