Gulin Oz1, Diane Hutter1, Ivan Tkac1, Harry T. Orr1, Khalaf Bushara1, Christopher M. Gomez2
1University of Minnesota, Minneapolis, MN, USA; 2University of Chicago, Chicago, IL, USA
To determine how similar neurochemical abnormalities as measured by high field MRS are in patients and model mice with the same genetic defect, we measured cerebellar neurochemical profiles of patients with SCA1, as well as of a mouse model of SCA1 relative to controls. The neuronal markers NAA and glutamate were decreased and the putative gliosis marker myo-inositol was increased in both mice and patients with SCA1. These data indicate that findings regarding neurochemical changes as monitored by high field 1H MRS in future pre-clinical trials with SCA1 mice can be utilized for the design of clinical trials with patients.