Misprocessing of canonical histone transcripts in a subset of Aicardi–Goutières syndrome (AGS) patients with biallelic mutations in LSM11 and RNU7-1 specifically disturbs linker histone stoichiometries and leads to type I IFN signatures by cGAS redistribution and activation within the nucleus (Uggenti et al., 2020). The gene discussed is RNU7-1; the disease is Aicardi-Goutieres syndrome.