with MWS. While the mechanism underlying epilepsy in individuals with ZEB2 mutations is not well understood, studies by McKinsey and Van den Berghe (McKinsey et al., 2013; van den Berghe et al., 2013) show the influence of ZEB2 on the neurogenesis of cortical γ-aminobutyric acid (GABA)ergic interneurons. Further, lack of ZEB2 prevents the repression of NKX2-1 homeobox transcription factor, the expression of which induces the differentiation of progenitor cells into striatal interneurons rather than cortical neurons (McKinsey et al., 2013; van den Berghe et al., 2013). Subsequently, deficit of GABAergic inhibition is thought to result in seizures (Yalçın, 2012). A recent exome sequencing study identified a de novo missense variant in ZEB2 and early infant epileptic encephalopathy characterized by burst-suppression EEG (Babkina et al., 2016). In addition, independent genetic studies have also found an association of ZEB2 variants, obesity related traits (Comuzzie et al., 2012), depression, bipolar disorder, and schizophrenia (Ripke et al., 2014). Taken together, this may suggest that variation in ZEB2 may have broader neurobiological implications, beyond epilepsy. While no previous genetic association studies have provided a clear association of either ZEB2 or CTBP2 and beta EEG, mRNA expression in key brain regions for beta EEG, along with
