A mystery long associated with Huntington’s disease has been resolved by a team of researchers at the UCSF-affiliated Gladstone Institute of Neurological Disease, thanks to a specially designed microscope that allows researchers to track changes in cells, including those associated with neurodegeneration, over long lengths of time.As reported in the cover story in the current issue of Nature (Oct. 14, 2004), the team determined that abnormal deposits of mutant huntingtin protein, which appear in the brains of all Huntington’s disease patients, aren’t the cause of neuronal death. Scientists know that mutant huntingtin protein is responsible for the disease, but they have not known in what form it wreaks its havoc. They haven’t known, for instance, whether the abnormal deposits of the protein, known as “inclusion bodies,” were, themselves, causative, protective or incidental to the disease. In the current study, the Gladstone team determined that inclusion bodies are a beneficial coping response, possibly sequestering mutant huntingtin protein, thereby reducing levels of the protein elsewhere in the neuron, and thus prolonging neurons’ survival.
