Matthew Weston, Ph.D.
"We’re examining the genes, cells, and critical time points in development that influence synaptic changes underlying epilepsy to find new, targeted therapies.”
Seeking solutions to childhood seizure disorders
How do certain genetic variants cause severe epileptic disease?
Recent next-generation DNA sequencing efforts in humans have uncovered a handful of genes in which loss- or gain-of-function variants cause severe neurodevelopmental disorders called developmental epileptic encephalopathies (DEEs). DEEs are characterized by unremitting, treatment-resistant seizures and intellectual disability. The fact that these DEE-causing variants cause severe epileptic disease suggests that they are exceptional cases that overcome the brain’s innate plasticity mechanisms. Matt Weston seeks to understand how and why these genetic variants cause such an extreme imbalance of excitation and inhibition in the brain. His premise is that this work will advance understanding of normal physiological processes, delineate disease mechanisms, and point toward novel therapeutic strategies.
- Associate Professor, Fralin Biomedical Research Institute at VTC
- Associate Professor, School of Neuroscience, College of Science, Virginia Tech
Shore, A. N., Li, K., Safari, M., Qunies, A. M., Spitznagel, B. D., Weaver, C. D., Emmitte, K., Frankel, W., & Weston, M. C. (2024). Heterozygous expression of a Kcnt1 gain-of-function variant has differential effects on somatostatin- and parvalbumin-expressing cortical GABAergic neurons. eLife, 13, RP92915. https://doi.org/10.7554/eLife.92915
Jones, D. J., Soundararajan, D., Taylor, N. K., Aimiuwu, O. V., Mathkar, P., Shore, A., Teoh, J. J., Wang, W., Sands, T. T., Weston, M. C., Harper, S. Q., & Frankel, W. N. (2024). Effective knockdown-replace gene therapy in a novel mouse model of DNM1 developmental and epileptic encephalopathy. Molecular Therapy : The Journal of the American Society of Gene Therapy, 32(10), 3318–3330. https://doi.org/10.1016/j.ymthe.2024.08.009
Cullen, E. R., Safari, M., Mittelstadt, I., & Weston, M. C. (2024). Hyperactivity of mTORC1- and mTORC2-dependent signaling mediates epilepsy downstream of somatic PTEN loss. eLife, 12, RP91323. https://doi.org/10.7554/eLife.91323
University of Vermont, Robert Larnder, M.D., College of Medicine, Assistant Professor
Baylor College of Medicine, Postdoctoral Fellow
Baylor College of Medicine, Postdoctoral Associate
- Baylor College of Medicine, Ph.D., Neuroscience
- University of Virginia, B.A., Echols Interdisciplinary Studies, concentration in Writing, English, German
- Professor John J. Trentin Scholarship award for Outstanding Coursework, 2005
- Rush and Helen Record Neuroscience Fellowship for Outstanding Graduate Student at Baylor College of Medicine, 2007-2008
- Coming Together on Epilepsy Genetics Meeting, Travel Award Jackson Labs, Bar Harbor, ME, 2011
- Fellow, NIH-NINDS Brain Disorders and Development Training Grant, 2010-2012
- Epilepsy Foundation Postdoctoral Research Fellowship, 2013-2014
- American Epilepsy Society Young Investigator Workshop Invited Speaker and Travel Award, 2014
- Citizens United for Research in Epilepsy (CURE) Young Investigator Travel Award GRC, 2014
- K99 Pathway to Independence Award, NIH/NINDS, 2014
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