Recent studies have identified, in more than 75% of DIPG cases, a somatic mutation of the H3F3A gene, leading to a lysine 27 to methionine mutation at histone H3 variant H3.3 (H3.3K27M) (Castel et al., 2015; Schwartzentruber et al., 2012; Sturm et al., 2012; Wu et al., 2012, 2014). Here, H3C3 is linked to diffuse intrinsic pontine glioma.