PROJECT 5:

Host:

Laboratoire d' Enzymologie et Biochimie Structurales - UPR 3082 CNRS

 

PI:
Ronald Melki

ESR:
Gemma Ruiz Arlandis

Huntington’ disease (HD) is an inherited neurodegenerative disorder defined by progression of neuronal dysfunction and death of neurons. HD is induced by a polyglutamine expansion characterized by repeats of the triplet CAG located in the Exon-1 of Huntingtin gene. The disease appears when the number of repeats is higher than 35, and the PolyQ expansion makes the soluble protein form cytotoxic aggregates called amyloids. Those structures have the ability to propagate between cells and may contribute to the evolution of deleterious neurodegeneration. Our research area is focused on the mechanisms that allow the aggregates to bind and penetrate the cells, their movement inside the cytosol, the cell compartments they are able to reach and the functions they affect according to their location.

For this approaches, we are using different techniques such as Epifluorescence microscopy, live cell imaging, confocal microscopy, Total Internal Reflection Microscopy (TIRF) and Electronic microscopy.