This role of caspase-6 may have disease relevance as caspase-6 has been linked to Alzheimer’s (Gervais et al., 1999; Guo et al., 2004; Albrecht et al., 2007) and to Huntington’s disease (Graham et al., 2006; Graham et al., 2010). Here, CASP6 is linked to juvenile Huntington disease.