Furthermore, MSH3 may be a shuttling protein as well, a feature that is highly relevant in this context, as is the discovery of agents that either promote or prevent nuclear import of MutSβ (MSH2-MSH3 heterodimer) which have been investigated to treat trinuleotide repeat expansions that drive Huntington’s disease (HD) and other severe genetic diseases. Here, MSH3 is linked to juvenile Huntington disease.