Recent studies have revealed that EZH inhibitory protein EZHIP (previously known as CXorf67), which is aberrantly expressed in most PFA ependymomas (and mutated in some), causes downregulation of H3K27me3 by inhibiting EZH2 in the polycomb repressive complex 2 (PRC2)10,11. This evidence concerns the gene EZHIP and ependymoma.