by Tony Fitzpatrick
Huntington’s disease is cruel and devastating. The inherited disorder’s signature is the wasting away of brain nerve cells, leading to a host of nightmarish symptoms and outcomes. In Huntington’s, a portion of DNA known as a CAG repeat occurs 30 to 120 times rather than the 10 to 28 times that it does in normal cells. As the gene passes through families, the CAG repeats often get longer, hastening the development of disease at increasingly younger ages. Symptoms include uncontrolled movements, fidgeting, hallucinations, paranoia and dementia. One in 10,000 people of European stock is affected by Huntington’s. There is no cure, and while some drugs show promise, no known way exists to prevent the disease from getting worse.
, PhD, professor of biomedical engineering and director of the new Center for Biological Systems Engineering, studies proteins involved in the development of Huntington’s disease and related neurodegenerative motor control disorders. All involve an ensemble of recently recognized eccentric proteins, known as intrinsically disordered proteins (IDPs), and share the common theme of protein aggregation, or clumping, leading to neuronal death and disease. Perhaps the best-known example of protein aggregation is the beta amyloid plaques seen in the brains of Alzheimer’s disease patients.