![Cracking Huntington’s Disease]()
A paper by Mark Mehler, M.D., published in Proceedings of the National Academy of Science USA (PNAS), found that when the pathogenic protein, mutant huntingtin (mHtt), was expressed solely during embryonic development it can trigger characteristic features of Huntington’s disease (HD), an inherited neurodegenerative disorder (NDD). Symptoms for HD often do not develop until middle age. However, the researchers found that mice with selective developmental expression of mHtt exhibited hallmarks of the disease, including neurodegeneration, impaired motor coordination, and electrophysiological impairments in awake, moving mice These impairments include altered synaptic plasticity and neural connectivity, which indicate impaired learning and memory, and improper brain wiring, respectively. These findings may help to explain previous reports indicating that more subtle brain deficits precede disease onset in humans. The findings could also lead to novel biomarkers and innovative therapeutic strategies for preventing Huntington’s disease with important implications for other NDDs. Dr. Mehler is professor and chair of the Saul R. Korey Department of Neurology and director of the Institute for Brain Disorders and Neural Regeneration. He is also professor of neuroscience and of psychiatry and behavioral sciences, and holds the Alpern Family Foundation Chair in Cerebral Palsy Research.
Posted on: Friday, May 27, 2016