A team of scientists at the University of California, San Francisco (UCSF) has developed a new model for how inherited genes contribute to a common but untreatable and incurable neurodegenerative disease. The disease, frontotemporal lobar degeneration, is the second most common cause of dementia before age 65, after Alzheimer's disease.
Based on experiments in worms and mice, the UCSF team's work explains in part why the brain deteriorates in frontotemporal lobar degeneration, which may have implications for the understanding of several neurodegenerative disorders, including Alzheimer's and Parkinson's, as well as different forms of cancer.
"If our findings hold up," said Aimee Kao, an assistant adjunct professor in the Department of Neurology at UCSF, "they may suggest a new way to think about how to treat neurodegenerative diseases." Kao is first author on the study, led by Cynthia Kenyon, PhD, a professor of biochemistry and biophysics at UCSF and director of UCSF's Larry L. Hillblom Center for the Biology of Aging.
Disease Caused By Loss of Neurons
Generally scientists have blamed the mental decline associated with neurodegenerative diseases on the loss of neurons associated with the accumulation of insoluble protein in the brain sticky plaques that interfere with and ultimately kill the brain's neurons.
In frontotemporal lobar degeneration, this loss of neurons happens in the frontal lobe the part of the brain involved in such higher mental functions as art appreciation and emotional empathy. People with this disease can suffer from progressive difficulties with language, undergo personality and behavioral changes, and usually die within a decade of diagnosis.
The new work suggests that the accumulation of insoluble protein may not be the only cause of cognitive decline in frontotemporal lobar degeneration. Another mechanism could involve how the body deals with injured neurons in the brain.
|Contact: Jason Socrates Bardi|
University of California - San Francisco