SpinoCerebellar Ataxia Type 7 (SCA7) is an autosomal dominant degenerative disease defined by neurodegeneration of the retina and cerebellum. While gene silencing therapeutic strategy is developing, there is a need of biomarkers for evaluation of their efficacy. Here we characterize a recent knock-in mouse model of SCA7 with in-vivo longitudinal MRI and MRS. SCA7140Q/5Q display a wide range of phenotypes, including morphological and metabolic alterations in key brain structures. Our longitudinal protocol allowed better understanding of the chronology of these alterations and offers pertinent biomarkers for evaluation of therapies using the SCA7140Q/5Q model.
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