Dr. Jianning Wei

Jianning Wei, Ph.D., assistant professor of biomedical sciences in the Charles E. Schmidt College of Biomedical Science at FAU, has received a R15 grant from the National Institutes of Health (NIH) to further her research into the molecular mechanisms of Huntington's disease (HD). HD is highly complex genetic, neurological disorder that causes certain nerve cells in the brain to waste away. It is a fatal inherited disease, characterized by a selective loss of neurons in the brain, affects the basal ganglia, which controls motor control, cognition, learning and emotions, as well as the outer surface of the brain, or the cortex, which controls thought, perception, and memory.

Wei and her colleagues are working to identify the pathways in the brain that are altered in response to mutant proteins, as well as to understand the cellular processes impacted by the disease in order to facilitate the development of effective pharmacological interventions.

The underlying molecular mechanism of HD remains elusive. Wei hopes that the information obtained from the studies will improve the current understanding of the molecular pathways that are altered in response to mutant huntington or mHtt. Wei's findings may also represent a universal mechanism in the pathogenesis of neurodegenerative diseases that are involved with protein misfolding and aggregation. Preliminary data from their findings using an in vitro cell model of HD suggest that there is a novel mechanism for mHtt induced cell death, or apoptosis. Apoptosis has been proposed as one of the mechanisms leading to neuronal death in HD. With this NIH grant, Wei and her colleagues will be testing their hypothesis in a mouse model of HD.

HD and symptoms don't appear until middle age. In addition to uncontrolled movements, neurological degeneration also causes loss of intellectual facilities and emotional disturbance, and as the disease progresses can affect a person's ability to walk, talk and swallow. Today, approximately 30,000 Americans are living with HD. In addition, a staggering 200,000 more are at risk. Although physicians may prescribe a number of medications to help control emotional and movement problems associated with HD, there is no treatment to stop or reverse the course of the disease.