Similarly, Hoeman et al recently characterized a novel genetic mouse model of DIPG, demonstrating brainstem glioma formation with ACVR1 R206H or G328 V, H3.1K27 M, and p53 deletion, but not with ACVR1 or H3.1K27 M mutations alone [34]. Here, ACVR1 is linked to diffuse intrinsic pontine glioma.