Habib et al. (2014), through a study with 57 patients with BWS, demonstrated that some patients who present methylation gain in the H19/IGF2 locus also present mutations in the binding site of the OCT4/SOX2 factors, showing that the SOX/OCT motifs within H19/IGF2 ICR also participate in maintaining hypomethylation of the maternal allele [29]. The gene discussed is IGF2; the disease is Beckwith-Wiedemann syndrome.