Though the complete removal of 3'-5'-DNA exonuclease coded by cg3165 is rather attributable to Aicardi-Goutieres syndrome (38), and is not relevant to the RVCL-S caused by the active enzyme mis-localization, the results obtained with the deletion line were used as a starting point to assess the phenotypic impacts from human TREX1 transgenes. This evidence concerns the gene TREX1 and Aicardi-Goutières syndrome.