Dysregulation Of Non-Coding Rna Expression In Huntington Disease Model Mouse

Background: Huntington disease (HD) is an inherited neurodegenerative disorder that causes chorea, psychiatric disturbances and dementia. In the brains of HD patients, neurodegeneration is most pronounced in the striatum, and medium spiny neurons (MSN) are lost. It is known that HD is caused by expanded CAG repeats encoding polyglutamine in the exon1 of Huntingtin (Htt). In the pathological situation, the N-terminal fragment of mutant Htt forms neuronal intranuclear inclusions in the brain and disrupts the activities of transcriptional machinery such as transcription factors. According to some previous studies, the expressions of some non-coding RNAs (ncRNAs) are dysregulated in HD patients.