Dr. Benoit Gentil

Motor development; Rare neurological disease; Genetics and epigenetics; Neurobiology; stress and stress proteins.
Students interested in pursuing training opportunities are encouraged to contact Dr. Gentil directly,providing a copy of their current resumé or curriculum vitae.
- Postdoctoral Fellow, mentor: Walter Mushynski, dep. of biochemistry, McGill University,
- Postdoctoral Fellow, mentor: Jean-pierre Julien, dep. of neurology and neurosurgery, McGill University,
- Ph.D., Molecular and Cell Biology. Université Joseph Fourier, Grenoble.
- MSc in Human Nutrition, Diplôme d’Etudes Approfondies in Physiology and Pathology of Human Nutrition, University Diderot (Paris VII).
- Licence (BSc) in Biology and Physiology, University Joseph Fourier, Grenoble.
Dagher R*, Massie R, Gentil BJ. MTP deficiency caused by HADHB mutations: Pathophysiology and clinical manifestations.Mol Genet Metab. 2021 May;133(1):1-7
A. Dabbaghizadeh*, A. Paré*, Z. Cheng-Boivin*, R. Dagher*, S. Minotti**, M.Dicaire, B. Brais, J. Young, H.D. Durham, B.J. Gentil. The J domain of sacsin disrupts intermediate filament assembly. Bio Rxiv 2021.
D.L. Demy, M.L. Campanari, R. Munoz-Ruiz, H.D. Durham, B.J. Gentil, E. Kabashi, Functional Characterization of Neurofilament Light Splicing and Misbalance in Zebrafish, Cells, 9(2020).
R. Kuta, N. Larochelle, M. Fernandez, A. Pal, S. Minotti, M. Tibshirani*, K. St Louis, B.J. Gentil, J.N. Nalbantoglu, A. Hermann, H.D. Durham, Depending on the stress, histone deacetylase inhibitors act as heatshock protein co-inducers in motor neuronsand potentiate arimoclomol, exerting neuroprotection throughmultiple mechanisms in ALS modelsCell Stress Chaperones 25 (2020) 173-191.
J.Baudier, B.J. Gentil, The S100B Proteinand Partners in Adipocyte Responseto Cold Stressand Adaptive Thermogenesis: Facts, Hypotheses, and Perspectives. Biomolecules 10 (2020).
B.J. Gentil, G.T. Lai *, M. Menade,R. Lariviere, S. Minotti**, K. Gehring, J.P. Chapple, B. Brais, H.D. Durham, Sacsin, mutated in the ataxia ARSACS, regulates intermediate filament assembly and dynamics FASEB J 33 (2019) 2982-2994.
M. Tibshirani, B. Zhao, B.J. Gentil, S. Minotti, C. Marques, J. Keith, E. Rogaeva, L. Zinman, C. Rouaux, J. Robertson, H.D. Durham, Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis Hum Mol Genet 26 (2017) 4142-4152.
B.J. Gentil, E. O'Ferrall, C. Chalk, L.F. Santana, H.D. Durham, R. Massie, A New Mutationin FIG4 Causes a SevereForm of CMT4J Involving TRPV4 in the Pathogenic CascadeJ Neuropathol Exp Neurol 76 (2017) 789-799.
E.J. Duncan, R. Lariviere, T.Y. Bradshaw, F. Longo, N. Sgarioto, M.J. Hayes, L.E.L. Romano,S. Nethisinghe, P. Giunti, M.B. Bruntraeger, H.D. Durham, B. Brais, F. Maltecca, B.J. Gentil, J.P. Chapple,Altered organization of the intermediate filament cytoskeleton and relocalization of proteostasis modulators in cells lacking the ataxia protein sacsin Hum Mol Genet 26 (2017) 3130-3143.