Dion is interested in the identification of novel genetic risk factors in a range of neurological conditions (amyotrophic lateral sclerosis, essential tremor, restless legs syndrome) as well as into the characterization of the underlying pathogenic events associated with these risk factors. His examinations of the nuclear pore component, Gle1, in ALS opened the door to a range of examination of nuclear-cytoplasmic export defect where Gle1 is mislocalized.