Huntington's Disease

Huntington’s disease (HD) is an inherited neurological disorder that causes progressive loss of motor function, cognitive decline and emotional disturbances. The cascade of events that cause the selective loss of neurons in a brain region called the striatum is not well understood. Transgenic mouse models of HD have emerged as a powerful tool to investigate the pathophysiology of this disease. Using ultra-high field MRS CMRR researchers study the neurochemical changes in transgenic mouse models of HD. Methodology developed at CMRR (9,10) enables reliable quantification of 15 brain metabolites from volumes as small as 4 - 5 microliters (a volume with dimensions of 1-2 mm) (11). This noninvasive method is ideal for investigating neurochemical changes in the progress of HD longitudinally (Figures 4 and 5) and provides insights into the underlying cellular changes in HD.