The significantly elevated abundances of STAT1, DDX58, and ISG15 in the brains of 3-month-old ClpP-null mice demonstrated this inflammation in nervous tissue to precede neural phenotypes; in the mouse model of Perrault syndrome, the neurodegeneration features such as hearing loss, ataxia, and white matter degeneration do not appear before ages around 12 months [10]. Here, ISG15 is linked to Perrault syndrome.