PMID- 30098714 OWN - NLM STAT- MEDLINE DCOM- 20190308 LR - 20230804 IS - 1558-4410 (Electronic) IS - 0889-8529 (Print) IS - 0889-8529 (Linking) VI - 47 IP - 3 DP - 2018 Sep TI - Molecular Genetic Studies of Pancreatic Neuroendocrine Tumors: New Therapeutic Approaches. PG - 525-548 LID - S0889-8529(18)30519-X [pii] LID - 10.1016/j.ecl.2018.04.007 [doi] AB - 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. CI - Copyright (c) 2018 Elsevier Inc. All rights reserved. FAU - Stevenson, Mark AU - Stevenson M AD - Radcliffe Department of Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism (OCDEM), University of Oxford, Churchill Hospital, Headington, Oxford OX3 7LJ, UK. FAU - Lines, Kate E AU - Lines KE AD - Radcliffe Department of Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism (OCDEM), University of Oxford, Churchill Hospital, Headington, Oxford OX3 7LJ, UK. FAU - Thakker, Rajesh V AU - Thakker RV AD - Radcliffe Department of Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism (OCDEM), University of Oxford, Churchill Hospital, Headington, Oxford OX3 7LJ, UK. Electronic address: rajesh.thakker@ndm.ox.ac.uk. LA - eng GR - WT_/Wellcome Trust/United Kingdom GR - G1000467/MRC_/Medical Research Council/United Kingdom GR - G9825289/MRC_/Medical Research Council/United Kingdom PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - Endocrinol Metab Clin North Am JT - Endocrinology and metabolism clinics of North America JID - 8800104 RN - 0 (MEN1 protein, human) RN - 0 (Proto-Oncogene Proteins) SB - IM MH - Humans MH - Multiple Endocrine Neoplasia Type 1 MH - Neuroendocrine Tumors/*genetics/*therapy MH - Pancreatic Neoplasms/*genetics/*therapy MH - Proto-Oncogene Proteins/genetics PMC - PMC7614857 MID - EMS181859 OTO - NOTNLM OT - Epigenetic OT - MEN1 OT - Menin OT - PNETs OT - RAS OT - SSTRs OT - VHL OT - mTOR COIS- Disclosure Statement: The authors have no conflicts of interest to disclose. EDAT- 2018/08/14 06:00 MHDA- 2019/03/09 06:00 PMCR- 2023/08/01 CRDT- 2018/08/13 06:00 PHST- 2018/08/13 06:00 [entrez] PHST- 2018/08/14 06:00 [pubmed] PHST- 2019/03/09 06:00 [medline] PHST- 2023/08/01 00:00 [pmc-release] AID - S0889-8529(18)30519-X [pii] AID - 10.1016/j.ecl.2018.04.007 [doi] PST - ppublish SO - Endocrinol Metab Clin North Am. 2018 Sep;47(3):525-548. doi: 10.1016/j.ecl.2018.04.007.