PMID- 30781283 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20191120 IS - 1744-8417 (Electronic) IS - 1744-6651 (Linking) VI - 4 IP - 4 DP - 2009 Jul TI - Multiple endocrine neoplasia type 1. PG - 371-388 LID - 10.1586/eem.09.22 [doi] AB - Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal-dominant inherited tumor syndrome characterized by hyperplasia and/or tumors in the parathyroid glands, the pancreatic islets, the anterior pituitary and adrenal glands, as well as neuroendocrine tumors in the thymus, lungs and stomach, and tumors in nonendocrine tissues. In 1997, the responsible MEN1 gene was identified as a tumor-suppressor gene and its product was named menin. In this review, guidelines for early diagnosis, including MEN1 gene mutation analysis, and treatment, including periodic clinical monitoring, have been formulated, enabling improvement of life expectancy and quality of life. Identification of menin-interacting proteins has provided new insights into the function of menin, notably involving regulation of gene transcription related to proliferation and apoptosis, genome stability and DNA repair, and endocrine/metabolic homeostasis. In the near future, target-directed intervention may prevent or delay the onset of MEN 1-related tumors. FAU - Lips, Cornelis Jm AU - Lips CJ AD - a University Medical Center Utrecht, Department of Internal Medicine, Wassenaarseweg 109, 2596 CN The Hague, The Netherlands. lips05@zonnet.nl. FAU - Dreijerink, Koen AU - Dreijerink K AD - b University Medical Center Utrecht, Department of Internal Medicine, F02.126, PO Box 85500, 3508 GA, Utrecht, The Netherlands. k.m.a.dreijerink@umcutrecht.nl. FAU - Links, Thera P AU - Links TP AD - c University Medical Center Groningen, Department of Internal Medicine, PO Box 30001, 9700 RB Groningen, The Netherlands. t.p.links@int.umcg.nl. FAU - Hoppener, Jo Wm AU - Hoppener JW AD - d Department of Metabolic and Endocrine Diseases, PO Box 85090, 3508 AB Utrecht. j.w.m.hoeppener@umcutrecht.nl. LA - eng PT - Journal Article PL - England TA - Expert Rev Endocrinol Metab JT - Expert review of endocrinology & metabolism JID - 101278293 OTO - NOTNLM OT - DNA diagnosis OT - MEN 1 OT - genetic testing OT - menin OT - multiple endocrine neoplasia type 1 OT - mutation analysis OT - periodic clinical monitoring OT - presymptomatic treatment EDAT- 2009/07/01 00:00 MHDA- 2009/07/01 00:01 CRDT- 2019/02/21 06:00 PHST- 2019/02/21 06:00 [entrez] PHST- 2009/07/01 00:00 [pubmed] PHST- 2009/07/01 00:01 [medline] AID - 10.1586/eem.09.22 [doi] PST - ppublish SO - Expert Rev Endocrinol Metab. 2009 Jul;4(4):371-388. doi: 10.1586/eem.09.22.