title: Epigenetic mechanisms drive the progression of neurofibromas to malignant peripheral nerve sheath tumors.
abstract: The polycomb repressive complex 2 (PRC2) is a histone methyltransferase complex known to repress gene expression. There is a large body of experimental evidence that supports its role in promoting tumorigenicity by suppressing tumor suppressor genes. Here, we discuss the surprising findings that, in neurofibromas, it may have a completely different role as a tumor suppressor; mutations of PRC2 lead to conversion of benign neurofibromas into malignant peripheral nerve sheath tumors (MPNSTs) by de-repressing and thereby activating genes driving cell growth and development. These findings have potentially powerful clinical applications in both diagnosing and treating MPNSTs.
  
  
  OBJECTIVE
  PRC2 loss drives malignant transformation of neurofibromas.
authors.affiliation: Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
authors.forename: Krish
authors.initials: K
authors.lastname: Suresh
authors.affiliation: Medical School for International Health, Beersheba, Israel.
authors.forename: Tamara
authors.initials: T
authors.lastname: Kliot
authors.affiliation: Department of Biochemistry and Molecular Genetics, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
authors.forename: Andrea
authors.initials: A
authors.lastname: Piunti
authors.affiliation: Department of Neurological Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
authors.forename: Michel
authors.initials: M
authors.lastname: Kliot
keywords: Epigenetics; MPNST; PRC2; neurofibroma