UCLA researchers identify molecular ‘switch’ that causes Huntington’s disease–like symptoms in mice

This study makes clear that a major neuroprotective function of N17 is to prevent the mutant protein from entering the nucleus and eliciting more severe toxicities,” Yang said, adding that the result is consistent with findings from several studies of other, related disorders in which mutant proteins with expanded glutamine in the nucleus are key for jump-starting a disease. The researchers also found that the mice in the study experienced inflammation in the brain somewhat similar to that found in people with Huntington’s. “Neuroinflammation is emerging as a potentially shared mechanism in multiple neurodegenerative disorders, including Alzheimer’s disease, Parkinson’s disease and amyotrophic lateral sclerosis, and the mouse model shows similar inflammation to that found in both Huntington’s and Alzheimer’s,” Yang said. “So these mice may have the potential to be used to study disease mechanisms and test neuroprotective or anti-inflammatory therapeutics for these various disorders.” Next, the researchers will focus on understanding the detailed molecular pathways that function through N17 to regulate trafficking the huntingtin protein between the cytoplasm and nucleus of a cell, and test whether targeting these pathways could prevent the onset or slow the progression of the disease in mouse models of Huntington’s. The study’s other authors were Jeffrey Cantle, Erin Greiner, C.Y. Daniel Lee, Albert Barth, Fuying Gao, Chang Sin Park, Susanna Sandoval-Mi...
Source: UCLA Newsroom: Health Sciences - Category: Universities & Medical Training Source Type: news