Séverine BOILLÉE
PhD, Team Leader, PI, INSERM
Team presentation
The team led by Séverine BOILLEE is interested in the mechanisms that induce motoneuron degeneration in ALS and that may result from a deleterious interaction between these neurons and microglial and macrophage cells (immune system cells).
The team’s goal is to identify pathways as therapeutic targets for slowing disease progression through 2 approaches:
- To understand how mutations in known or identifiable genes cause motoneuron death and microglial dysfunction.
- Discovering new therapeutic targets related to the immune system and the periphery (rather than the central nervous system (easier to access than the brain) by modulating the response of peripheral macrophages to spinal cord motoneuron degeneration.
Main publications
- Chiot A., Zaïdi S., Iltis C., Ribon M., Berriat F., Schiaffino L., Jolly A., de la Grange P., Mallat M., Bohl D., Millecamps S., Seilhean D., Lobsiger C.S., Boillée S. Modifying macrophages at the periphery has the capacity to change microglial reactivity and toextend ALS survival. Nat Neurosci. In press.
- Amador M.d.M., Muratet F., Teyssou E., Banneau G., Danel-Brunaud V., Allart E., Antoine J.-C., Camdessanché J.-P., Anheim M., Rudolf G., Tranchant C., Fleury M.-C., Bernard E., Stevanin G. & Millecamps S. Spastic paraplegia due to recessive or dominant mutations in ERLIN2 can convert to ALS, Neurology: Genetics, 5 (6):e374 (2019).
- Teyssou E.*, Chartier L.*, Amador M.d.M., Lam R., Lautrette G., Nicol M., Machat S., Da Barroca S., Moigneu C., Mairey M., Larmonier T., Saker S., Dussert C., Forlani S., Fontaine B., Seilhean D., Bohl D., Boillée S., Meininger V., Couratier P., Salachas F., Stevanin G., Millecamps S. Novel UBQLN2 mutations linked to Amyotrophic Lateral Sclerosis and atypical Hereditary Spastic Paraplegia phenotype through defective HSP70- mediated proteolysis. Neurobiology of Aging, 58:239.e11-239.e20. (2017).
*equal contribution. - Mesci P., Zaïdi S., Lobsiger C.S., Millecamps S., Escartin C., Seilhean D., Sato H., Mallat M. & Boillée S. System xC- is a mediator of microglial function and its deletion slows symptoms in ALS mice. Brain, 138(Pt 1):53-68 (2015).
- Toli D., Buttigieg D., Blanchard S., Lemonnier T., Lamotte d’Incamps B., Bellouze S., Baillat G., Bohl D.*, Haase G.* Modeling amyotrophic lateral sclerosis in pure human iPSc-derived motor neurons isolated by a novel FACS double selection technique. Neurobiol. D
Team members
Séverine BOILLÉE
PhD, Team Leader, PI, INSERM
Delphine BOHL
PhD, PI, INSERM
Christian LOBSIGER
PhD, PI, INSERM
Stéphanie MILLECAMPS
PhD, PharmD, PI, CNRS