Several studies on microRNA pathogenic role in HD have been published in the last two decades, especially in postmortem analyses: besides the dysregulation of single micro-RNA, such as miR-9/9*, miR-132, miR-4488, miR-196a-5p, and miR-549a, among others (17–22), the alteration of the miRNome in HD CNS seems to be global because the mHTT affects the action of argonaute-2, potentially interfering with the biogenesis of all micro-RNA (23). Here, AGO2 is linked to Huntington disease.