Finally, the study of ATPase/helicase-deficient SNRNP200 variants that affect the antiviral response and the discovery of a mutant (C502A) that elicits an IFNB1 response independent of viral infection and fully rescues IFN-β in SNRNP200 KD cells (Fig 4C and S11 Fig) further support the SNRNP200 ATPase/helicase function in conferring specificity to viral RNA and preventing signaling through the recognition of self-RNA, as recently reported for the natural gain-of-function DDX58 and IFIH1 ATPase-deficient variants [29]. Here, DNAH8 is linked to viral infectious disease.