ISG15 (UCRP) and LMP2 pools were found to be basally elevated in human fibroblasts with the inherited rare neurodegenerative disease Ataxia Telangiectasia (A-T) due to constitutive activation of the IFN-β induction pathway, first demonstrated by Siddoo-Atwal et al. [65]. Here, ISG15 is linked to Ataxia-telangiectasia.