In MPNSTs, however, EZH2 has recurrently been identified intact, despite the high rate of mutation in other key PRC2 components, SUZ12 and EED. Wassef and colleagues revealed that only the combined loss of both EZH1 and EZH2 in immortalized plexiform neurofibroma-derived cells produced deregulatory effects similar to that observed with the sole absence of either EED or SUZ12 [26]. This evidence concerns the gene SUZ12 and plexiform neurofibroma.