Cellular and molecular mechanisms of limbic system pathology in Huntington disease

In this PhD project, we hypothesize that the mutant huntingtin gene leads to toxic accumulation of RNA and protein products in selectively sensitive areas in Huntington Disease (HD). We hypothesize that this causes early pathology in the limbic system and the development of non-motor features of HD. Similar pathogenic mechanisms may be active in the amyothrophic lateral sclerosis-frontotemporal dementia spectrum of disorders. The overall aims of the project are therefore to determine the cellular and molecular mechanisms of pathology in the limbic system in HD and if restoration of these changes can lead to a therapeutic benefit.