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Motor dysfunction and neurodegeneration in a C9orf72 mouse line expressing poly-PR

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https://www.ncbi.nlm.nih.gov/geo/query/acc.cgi?acc=GSE132108
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GFP-PR28 homozygous mice show decreased survival time, while the heterozygous mice show motor imbalance, decreased brain weight, loss of Purkinje cells and lower motor neurons, and inflammation in the cerebellum and spinal cord. Transcriptional analysis shows that in the cerebellum, GFP-PR28 heterozygous mice show differential expression of genes related to synaptic transmission. The mRNA profiles of cerebellum, cortex and spinal cord of control and heterozygous mice were generated by RNA sequencing, in triplicate, using Illumina HiSeq X Ten
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2019-07-16
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