Analysis on the transcriptomic profile in the mouse cerebellum with LINE-1 overexpression

We report the transcriptomic profile of the cerebella of the mice in which a class of endogenous retroelements long interspersed nuclear elements-1 (LINE-1) is overexpressed with CRISPR-a modules targeting the promoter sequence of evolutionally young LINE-1 family. Transgenic mice for guide RNA targeting the LINE-1 promoter sequence were generated, crossed with another transgenic mice for dCas9-CRISPR-a module. The double transgenic mice presented with strong cerebellar ataxia phenotype as well as robust overexpression of LINE-1 in the cerebellum. The result of transcriptomic profiling of the cerebella in these mice showed close similarities of the dysregulated gene profiles in human cerebellar ataxia patients' cerebella, suggesting the usefulness of these mice in the studies of the etiopathogenesis of human cerebellar ataxia. Overall design: Analysis on the RNA expression profile in the cerebellum of 8-wk-old mice in which LINE-1 is activated (LINE-1a mice) and of control mice (dCas mice).