In yeast, Nop58p depletion and the introduction of the PEHO syndrome-causing ZNHIT3 variants C11F and S29L (corresponding to p.Cys14Phe and p.Ser31Leu in human) result in lower levels of both rRNA modifying and processing box C/D snoRNAs.9 Here, ZNHIT3 is linked to PEHO-like syndrome.