- Brain Awareness Week
Neurological Birth Defects
This laboratory investigates the biological mechanisms underlying neurologic birth defects. We are specifically interested in the brain injuries induced by lymphocytic choriomeningitis virus (LCMV) and alcohol (fetal alcohol syndrome). In our studies of congenital LCMV infection, we utilize a rat model of the infection to study the cellular and molecular mechanisms underlying the virus-induced neuropathology. We are investigating the immune cell types and the cytokines involved in virus-induced neuronal death. In the developing brain, LCMV specifically infects certain neuronal populations and leaves others completely uninfected. We are studying the mechanisms by which LCMV spreads through the brain and are attempting to identify the metabolic and molecular characteristics of neurons that render them vulnerable to infection. Following infection with LCMV, both humans and the rats in our model system develop epilepsy. We are investigating the pathophysiology underlying this virus-induced epileptic condition.
In our studies of fetal alcohol syndrome, we utilize animal models and tissue culture systems to study the mechanisms of alcohol-induced brain injury and the anatomical, histological and behavioral consequences of alcohol exposure. We have recently developed a mouse model of fetal alcohol syndrome, in which we utilize knock-out mice to study the importance of specific genes in influencing the pathological and behavioral effects of alcohol exposure.
We have recently begun a new line of research focusing on development of gene therapy for Alexander Disease. Alexander Disease is a devastating neurological disease of children caused by an autosomal dominant mutation of glial fibrillary acidic protein (GFAP), a protein expressed in astrocytes of the central nervous system. We are developing viral gene therapy vectors for delivery of RNA inhibition to astrocytes to specifically turn off the mutated GFAP gene in astrocytes.