Inborn errors of OAS-RNase L in SARS-CoV-2-related multisystem inflammatory syndrome in children

Multisystem inflammatory syndrome in children(MIS-C)is a rare and severe condition that follows benign COVID-19.We report autosomal recessive deficiencies of OAS1,OAS2,or RNASEL in five unrelated children with MIS-C.The cytosolic dsRNA-sensing OAS1 and OAS2 generate 2'-5'-linked oligoadenylates(2-5A)that activate the ssRNA-degrading RNase L.