. Author manuscript; available in PMC: 2020 Jul 1.

Published in final edited form as: Mol Cancer Res. 2019 Apr 25;17(7):1417–1428. doi: 10.1158/1541-7786.MCR-19-0147

Table 2:

Selected genes and chromatin elements discussed in this review.

Abbreviation	Protein	Description
PRC2	Polycomb repressive complex 2	• Chromatin modifying complex responsible for laying down H3K27me3 • Contains SUZ12, EED, EZH2, and RBBP4/7 • Frequently disrupted in MPNST
SUZ12	Suppressor of zeste 12 homologue	• Core component of PRC2 • Frequently mutated in MPNST • Deleted in NF-1 microdeletion syndrome
EED	Embryonic ectoderm development protein	• Core PRC2 component • Frequently mutated in MPNST
EZH2	Enhancer of zeste homologue 2	• Core, catalytic PRC2 component • Mutations not observed in MPNST • Increased expression in a subset of MPNSTs
*CDKN2A*	Cyclin dependent kinase inhibitor 2A	• Gene encoding tumor suppressors p16^INK4A and p14^ARF • p14^ARF and p16^INK4A transcripts transcribed in different reading frames • Often lost or silenced in MPNST
p16^INK4A	Cyclin dependent kinase 4 inhibitor A	• A protein encoded by CDKN2A • Absent in approximately 75% of MPNSTs
H3K27me3	Histone H3 lysine 27 trimethylation	• Epigenetic mark deposited by PRC2 • Typically lost along with mutations PRC2 core components