Uzay Emrah Emir1, H Brent Clark1, Manda Vollmers1, Dee M. Koski1, Lynn E. Eberly1, Harry T. Orr1, Huda Y. Zoghbi2, Gulin Oz1
In order to identify MRS biomarkers of very early neurochemical changes related to neurodegeneration, we utilized a knockin (KI) mouse model of SCA1. We measured cerebellar neurochemical profiles of KI mice and wild-type (WT) littermates longitudinally at 9.4T using short-echo LASER. Total choline, taurine and glutamine were the most robust biomarkers in this model. The KI mice displayed very mild cerebellar pathology even at 9 months, however they were distinguished from WTs by MRS starting at 6 weeks. Therefore, this study demonstrated that the MRS biomarkers are sensitive to very early changes related to neurodegeneration prior to overt pathology.