Since RNase L is activated in the absence of ADAR1 editing contributing to cell death, RNase L-induced cell death could potentially contribute to symptoms of the neurodegenerative and inflammatory disease Aicardi-Goutiéres syndrome (AGS) in cases with mutation of ADAR1 (Rice et al., 2012). This evidence concerns the gene ADAR and Aicardi-Goutieres syndrome.