USP16 has been reported to contribute to the somatic stem-cell defects in DS and reduce the self-renewal of multiple somatic stem cells [17], suggesting that some of the pathological features associated with DS may result from a stem-cell imbalance due to overexpression of USP16. It was first identified as a histone H2A specific deubiquitinase that regulates cell cycle progression and gene expression in human cells [18]. The gene discussed is USP16; the disease is Dravet syndrome.