Atlas One · Host Biology · Scale 03 — Molecular

H3K27M

H3K27M is a histone H3 driver mutation (H3F3A or HIST1H3B K27M); dominant-negative inhibition of PRC2/EZH2 → global H3K27me3 loss; defines WHO Grade 4 diffuse midline glioma; ONC201 (imipridone) FDA-approved for H3K27M+ DMG; H3K27M IHC is diagnostic.

Also known as: H3K27M, H3.3K27M, H3F3A K27M, HIST1H3B K27M, histone H3.3 mutation glioma, DIPG H3K27M, diffuse midline glioma H3K27M, H3K27me3 loss glioma, H3 oncohistone, histone K27M mutation

Scale 03 — MolecularScale
draftStatus
2026-06-07Last Reviewed
This is an auto-generated stub page built from atlas entry frontmatter. A richer hand-crafted page is planned. See the atlas source for the full entry including detailed body sections.
h3k27m

H3K27M

H3K27M is a histone H3 driver mutation (H3F3A or HIST1H3B K27M); dominant-negative inhibition of PRC2/EZH2 → global H3K27me3 loss; defines WHO Grade 4 diffuse midline glioma; ONC201 (imipridone) FDA-approved for H3K27M+ DMG; H3K27M IHC is diagnostic.

Entry Metadata

FieldValue
IDh3k27m
NameH3K27M
Statusdraft
Last reviewed2026-06-07
Atlas01-human
Scale03-molecular

Cross-Atlas Connections

connects-toEzh2 connects-toCdkn2A connects-toPdgf connects-toMyc

Sources