Scientists have known for some time that striatal neurons selectively die in the brains of patients with Huntington disease (HD) and have assumed that this event is caused by a mutated version of a protein called huntingtin. Now, new research by an international team of investigators from Italy, Canada, the United States, and Sweden provides insight into the function of normal huntingtin protein, a finding that could point toward new treatment strategies for the disease.
The researchers discovered that huntingtin regulates the production of another protein, called brain-derived neurotrophic factor (BDNF), which is essential for survival of striatal neurons.
Stephenson J. Huntington Disease Clues. JAMA. 2001;286(2):159. doi:10.1001/jama.286.2.159-JWM10006-3-1