In mice, ZFP57 plays the predominant role in the maintenance of imprinting, whereas, in its absence, ZFP445 is required to preserve methylation in a subset of imprinting control regions such as those present in the MEG3/DLK1:IG-DMR, that involved in Temples’ syndrome. The gene discussed is ZFP57; the disease is motor developmental delay due to 14q32.2 paternally expressed gene defect.