Dietary restrictions eased symptoms and increased the life span of mice carrying the gene that causes Huntington's disease (HD), with implications for the treatment of humans with the disease, according to a new study.
HD is a hereditary neurodegenerative disorder affecting about one person in every 10,000 in the United States. The disorder causes deterioration of nerve cells, particularly in the striata and cerebral cortex, and is characterized by numerous neurological defects, such as motor, cognitive, and psychiatric abnormalities. In addition, patients with HD also suffer from hyperglycemia and tissue wasting. A mutation in the huntingtin protein causes HD; the normal function of the huntingtin protein and the manner in which the mutant form damages nerve cells remain unknown. However, the mutant huntingtin is associated with abnormalities in cellular energy metabolism and deficits in the amounts of two proteins involved in cellular resistance to stress—brain-derived neurotrophic factor (BDNF) and heat-shock protein-70 (HSP-70)—suggesting that neurons in HD have a reduced ability to cope with stress.
This is a preview of subscription content, access via your institution