Huntingtin aggregation cell cultures and mouse models as well as post-mortem samples from patients with Huntington’s disease have also shown that Argonaute-2 (AGO2), a core component of RISC, re-localizes and accumulates in the stress granules located in the striatal neurons that express mutant Huntingtin (mHTT) aggregates, thus hindering late autophagosome and lysosome fusion and, at the same time, leading to a global increase in miRNA expression [31]. Here, AGO2 is linked to juvenile Huntington disease.