Johnny Ngsee
Affiliate Investigator, Neuroscience
Ottawa Hospital Research Institute
Associate Professor, Cellular and Molecular Medicine
University of Ottawa
Research Interests
Dr. Ngsee is performing research on vesicle
trafficking and its role in neurodegenerative diseases. He is currently focused
on Amyotrophic Lateral Sclerosis (ALS; commonly referred to as “Lou Gehrig’s
disease”), a progressive neurodegenerative disease caused by death of motor
neurons. Cellular processes associated with familial ALS genes are diverse,
suggesting the etiology is multifactorial involving multiple biological
processes. Dr. Ngsee’s research focuses on VAPB (VAMP-associated protein B), a
gene mutated in an autosomal dominant, slow progressing form of ALS. His recent
study showed that mutant VAPB blocks protein trafficking in a membrane
compartment derived from the endoplasmic reticulum (ER) called the ER-Golgi
intermediate compartment (ERGIC). Mutant VAPB primarily affects retrograde
cargoes from ERGIC. Nuclear envelope proteins also utilize this transport route
and loss of VAPB disrupts their delivery that leads to progressive
deterioration of the nuclear envelope. His goals are to define the cellular
consequences of this nuclear envelope defect, characterize the biological
processes affected by the defect at ERGIC that could contribute to motor neuron
death, and explore treatment strategies that could mitigate the adverse effects
of mutant VAPB.
Brief Biography
Education:
1972-76 B.Sc.
(Honours) in Physiology, University of British Columbia
1976-78 M.Sc.
in Physiology, University of British Columbia, Dr. Nadine Wilson
1981-86 Ph.D.
in Biochemistry, University of British Columbia, Dr. Michael Smith
Research Experience:
1986 Postdoctoral
fellow, Pathology, University of British Columbia, Dr. Shirley Gillam
1987-93 Postdoctoral fellow, Molecular
and Cellular Physiology, Stanford University, Dr. Richard H. Scheller
1993-present Senior Scientist/ Associate Professor,
Ottawa Hospital Research Institute, University of Ottawa
Selected Publications
Pabba, M., A. Y. C. Wong, N. Ahlskog, E. Hristova, D. Biscaro, W. Nassrallah, J. K. Ngsee, M. Snyder, J. C. Beique, and Bergeron, R. NMDA receptors are upregulated and trafficked to the plasma membrane following sigma-1 receptor activation in the rat hippocampus. J. Neurosci. 34, 11325-11338 (2014).
Tran, D., A. Chalhoub, A. Schooley, W. Zhang, and J.K. Ngsee. A mutation in VAPB that causes amyotrophic lateral sclerosis also causes a nuclear envelope defect. J. Cell Sci. 125, 2831-2836 (2012).
Venderova, K., G. Kabbach, E. Abdel-Messih, Y. Zhang, R.J. Parks, Y. Imai, S. Gehrke, J.K. Ngsee, M.J. Lavoie, R. Slack, Y. Rao, Z. Zhang, B. Lu, M.E. Haque, and D.S. Park. Leucine-rich repeat kinase interacts with Parkin, DJ-1 and PINK-1 in a Drosophila melanogaster model of Parkinson's disease. Hum Mol Genet. 18, 4390-4404 (2009).
Prosser, D.C., D. Tran, P.Y.Gougeon, C. Verly, and J.K. Ngsee. FFAT rescues VAPA-mediated inhibition of ER-to-Golgi transport and VAPB-mediated ER aggregation. J. Cell Sci. 121, 3052-3061 (2008).
Hutt, D. M., Baltz,
J. M., and Ngsee,
J. K.
2005. Synaptotagmin VI and VIII and Syntaxin 2 are
Essential for the Mouse Sperm Acrosome Reaction. J. Biol. Chem. 280:
20197-20203
Diseases, conditions and populations of interest
Research and clinical approaches
