Interestingly, elevated p62 levels have been reported in model systems of neurodegenerative diseases including Huntington's disease (Gal et al., 2007; Nagaoka et al., 2004), and this may be sufficiently high to contribute to the accumulation of ubiquitinated substrates that has been recently reported in this condition (Bennett et al., 2007). This evidence concerns the gene SQSTM1 and juvenile Huntington disease.