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Abstract #0465

Detection of Early Neurochemical Changes Related to Neurodegeneration in a Spinocerebellar Ataxia Type 1 (SCA1) Mouse Model by 1H MRS at 9.4 Tesla

Uzay Emrah Emir1, H Brent Clark1, Manda Vollmers1, Dee M. Koski1, Lynn E. Eberly1, Harry T. Orr1, Huda Y. Zoghbi2, Gulin Oz1

1University of Minnesota, Minneapolis, MN, United States; 2Baylor College of Medicine, Houston, TX, United States

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.