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Pancreatic neuroendocrine tumors (PNETs) arise sporadically or as part of familial syndromes. Genetic studies of hereditary syndromes and whole exome sequencing analysis of sporadic NETs have revealed the roles of some genes involved in PNET tumorigenesis. The multiple endocrine neoplasia type 1 (MEN1) gene is most commonly mutated. Its encoded protein, menin, has roles in transcriptional regulation, genome stability, DNA repair, protein degradation, cell motility and adhesion, microRNA biogenesis, cell division, cell cycle control, and epigenetic regulation. Therapies targeting epigenetic regulation and MEN1 gene replacement have been reported to be effective in preclinical models.

Original publication

DOI

10.1016/j.ecl.2018.04.007

Type

Chapter

Publication Date

09/2018

Volume

47

Pages

525 - 548

Keywords

Epigenetic, MEN1, Menin, PNETs, RAS, SSTRs, VHL, mTOR, Humans, Multiple Endocrine Neoplasia Type 1, Neuroendocrine Tumors, Pancreatic Neoplasms, Proto-Oncogene Proteins