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Séminaire – Sandrine Humbert

vendredi 8 février 2019 / 11:30 - 13:00

Is Huntington disease –a late onset neurological condition- a neurodevelopmental disorder?

Sandrine Humbert

Sandrine Humbert
DR INSERM, team leader: Équipe « Progéniteurs neuraux et pathologies cérébrales » Grenoble Institut des Neurosciences – INSERM U836 – UGA

Abstract :

Huntington Disease (HD) belongs to the family of late onset manifesting neurological disorders including Alzheimer and Parkinson diseases. The cause of HD is the presence of an abnormal expansion of a polyglutamine tract in the huntingtin (HTT) protein. HD is characterized by a long premanifest phase before onset of progressive neurological and psychiatric symptoms at adult age, yet mutant HTT (mHTT) is expressed from the very beginning of life. 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.

Selected publications

Barnat M, Le Friec J, Benstaali C and Humbert, S (2017). Huntingtin-mediated Multipolar-Bipolar Transition of Newborn Cortical Neurons is Critical for their Postnatal Neuronal Morphology. Neuron, 93, 99-114.

Thion MS, McGuire JR, Sousa CM, Fuhrmann L, Fitamant J, Leboucher S, Vacher S, Tezenas du Montcel S, Bièche I, Bernet A, Patrick Mehlen P, Anne Vincent-Salomon A, and Humbert, S (2015). Unravelling the role of huntingtin in breast cancer metastasis. J. Natl. Cancer Inst., doi: 10.1093/jnci/djv208.

Elias S, McGuire JR, Yu H and Humbert S (2015). Huntingtin is required for epithelial polarity through RAB11A mediated apical trafficking of PAR3-aPKC. Plos Biol., 13:e1002142.

Molina-Calavita M, Barnat M, Elias S, Aparicio E, Piel M and Humbert S (2014). Mutant huntingtin affects cortical progenitor cell division and development of the mouse neocortex. J. Neurosci., 34, 10034-10040.

Elias S, Thion MS, Yu H, Moreira Sousa C, Lasgi C, Morin X and Humbert S (2014). Huntingtin Regulates Mammary Stem Cell Division and Differentiation. Stem Cell Reports, 2, 491-506.

Prochainement

  1. Séminaire – Suzana Herculano-Houzel

    vendredi 26 avril 2019 / 11:30

    Life slows down with more neurons in the cerebral cortex

  2. XIIIth IBAGS Meeting

    dimanche 28 avril 2019 - jeudi 2 mai 2019

    The XIIIth edition of the IBAGS meeting will be held in Biarritz from the 28th of April to the 2nd of May. In this meeting, we gather outstanding scientists and clinicians from all over the world to discuss about topics related to basal ganglia pathways, physiology, reinforcement learning and related disorders. Tutorials on basic science and clinical aspects of the basal ganglia will be given on the introducing day of the meeting. http://www.ibags2019.com/

  3. Séminaire impromptu – Maxime Assous

    vendredi 3 mai 2019 / 10:00 - 11:30

    Extrinsic innervation of striatal interneurons and implications for the local microcircuitry.

  4. Conférence mensuelle – Richard Robitaille

    vendredi 3 mai 2019 / 11:30 - 12:30

    Regulation of synaptic structure ad function by glial cells in health and disease

  5. Séminaire – Stéphanie Trouche

    vendredi 10 mai 2019 / 11:30 - 12:30

    Invitant : Catherine Le Moine

Détails

Date :
vendredi 8 février 2019
Heure :
11:30 - 13:00
Catégorie d’Évènement:

Lieu

CGFB
38 rue Albert Marquet
Bordeaux, 33000 France