Benoit Gentil

Dr. Benoit Gentil

Co-Applicant

Assistant Professor of Kinesiology and Physical Education at McGill University

Associate Member of Neurology and Neurosurgery at McGill University


NMD4C Involvement: Pillar 1: Preclinical Science

Email Benoit

Research Interests: Motor development, neurological disorders, genetic and epigenetics, Inherited Peripheral Neuropathies, CMT disease, ARSACS

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Biography

Dr. Benoit J Gentil is a Professor at McGill University, specializing in the field of neuromuscular disorders, In particular Charcot-Marie-Tooth disorder and Autosomal Recessive Ataxia of Charlevoix-Saguenay. With over two decades of experience in understanding the pathogenesis of these disease, he has gained a wealth of knowledge and experience that he uses to change lives through his research and teaching. 

Dr. Gentil holds a Ph.D of Molecular and Cell Biology from Grenoble University (France), and a Diplome d’Etude Approfondies in Human nutrition and Pathology from Diderot Université (Paris, France). Dr. Gentil is also actively involved in Muscular Dystrophy Canada, as a member of the Medical and Scientific Advisory Board, and in the Peripheral Nerve Society or Genome Quebec Network. His research focuses on the development of therapeutic approaches targeting the molecular mechanism of diseases. His work has helped to shed light on the underlying mechanisms of Peripheral Neuropathies as well as to contribute to the understanding of genetic factors affecting postnatal development. 


Recent Publications

Dabbaghizadeh, A, Paré, A, Cheng-Boivin, Z, Dagher, R, Minotti, S, Dicaire, MJ et al.. The J Domain of Sacsin Disrupts Intermediate Filament Assembly. Int J Mol Sci. 2022.23 (24) PMID:36555380

Dagher, R, Massie, R, Gentil, BJ. MTP deficiency caused by HADHB mutations: Pathophysiology and clinical manifestations. Mol Genet Metab. 2021.133 (1)1-7 PMID:33744096

Baudier, J, Gentil, BJ. The S100B Protein and Partners in Adipocyte Response to Cold Stress and Adaptive Thermogenesis: Facts, Hypotheses, and Perspectives. Biomolecules. 2020.10 (6) PMID:32486507

Demy, DL, Campanari, ML, Munoz-Ruiz, R, Durham, HD, Gentil, BJ, Kabashi, E et al.. Functional Characterization of Neurofilament Light Splicing and Misbalance in Zebrafish. Cells. 2020.9 (5) PMID:32429483

Kuta, R, Larochelle, N, Fernandez, M, Pal, A, Minotti, S, Tibshirani, M et al.. Depending on the stress, histone deacetylase inhibitors act as heat shock protein co-inducers in motor neurons and potentiate arimoclomol, exerting neuroprotection through multiple mechanisms in ALS models. Cell Stress Chaperones. 2020.25 (1)173-191 PMID:31900865

Gentil, BJ, Lai, GT, Menade, M, Larivière, R, Minotti, S, Gehring, K et al.. Sacsin, mutated in the ataxia ARSACS, regulates intermediate filament assembly and dynamics. FASEB J. 2019.33 (2)2982-2994 PMID:30332300

Tibshirani, M, Zhao, B, Gentil, BJ, Minotti, S, Marques, C, Keith, J et al.. Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis. Hum Mol Genet. 2017.26 (21)4142-4152 PMID:28973294

Gentil, BJ, O'Ferrall, E, Chalk, C, Santana, LF, Durham, HD, Massie, R et al.. A New Mutation in FIG4 Causes a Severe Form of CMT4J Involving TRPV4 in the Pathogenic Cascade. J Neuropathol Exp Neurol. 2017.76 (9)789-799 PMID:28859335

Duncan, EJ, Larivière, R, Bradshaw, TY, Longo, F, Sgarioto, N, Hayes, MJ et al.. Altered organization of the intermediate filament cytoskeleton and relocalization of proteostasis modulators in cells lacking the ataxia protein sacsin. Hum Mol Genet. 2017.26 (16)3130-3143 PMID:28535259

Picard, M, Azuelos, I, Jung, B, Giordano, C, Matecki, S, Hussain, S et al.. Mechanical ventilation triggers abnormal mitochondrial dynamics and morphology in the diaphragm. J Appl Physiol (1985). 2015.118 (9)1161-71 PMID:25767033

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