RNU7-1 and Aicardi-Goutieres syndrome: The biallelic mutations in LSM11 (U7 small nuclear RNA associated protein) and RNU7-1 (U7 small nuclear 1) encoding components of the replication-dependent histone pre-mRNA-processing complex have been detected in genetically uncharacterized cases of type I interferonopathy, Aicardi-Goutières syndrome (AGS) (83).