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Research Director CNRS Contact: florence.maschat(at)umontpellier.fr Phone: 33 (0)4 67 14 42 70 Team 4: Huntington’s disease: Neurophysiopathology from drosophila to mice Collaborations :
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Presentation
Our research topics include two axes.
One concerns the study of Engrailed homeoprotein. The identification of the regulatory networks involving this transcription factor have been carried out using genomic scale approaches: chromatin immunoprecipitation experiments to isolate direct targets genes and two-hybrid experiments in yeast to identify Engrailed cofactors. Our team has recently focused on Engrailed implication in neurodevelopment, and the identification of genes regulated by Engrailed and its cofactor (Neuro-Goosberry) to better understand the role of Engrailed in the construction of the nerve cord of an embryo.
On the other hand, among the direct targets of Engrailed, we identified that the Huntingtin, whose human homolog is involved in Huntington's disease (a rare neurodegenerative disease), is directly activated by Engrailed. This allowed us to develop a different axis and identify a 23aa peptide P42 contained in the Huntingtin protein which presents protective properties of this disease in Drosophila but also in mice. Besides obtaining international patents, P42 recently obtained an orphan drug designation by the European Medicines Agency (EMA). We plan to further develop this axis to better understand the physiological role of P42 and the mechanisms of action of this peptide in a view of its development at therapeutic purposes.