MEN1

Definition: MEN1 is a likely tumor suppressor gene that encodes a novel protein, menin. Menin is a 610 amino-acid residue protein with as yet unknown function(s).

Function

 menin associates with nonmuscle myosin II-A heavy chain (14508515)
 menin through binding to NMHC II-A could participate in cell division and in other processes that involve NMHC II-A (14508515)
 menin interacts with NF-kappaB proteins and inhibits NF-kappaB-mediated transactivation (11526476)
 menin interacts with the AP1 transcription factor JunD and represses JunD-activated transcription (9989505)

Pathology

 germline mutation in multiple endocrine neoplasia type 1 (MEN1)

 pancreatic neuroendocrine tumors

• Aberrant Menin expression is an early event in pancreatic neuroendocrine tumorigenesis. (27342911)
• Loss of ATRX or DAXX expression and concomitant acquisition of the alternative lengthening of telomeres phenotype are late events in a small subset of MEN-1 syndrome pancreatic neuroendocrine tumors. (22575867)

References

 Aberrant Menin expression is an early event in pancreatic neuroendocrine tumorigenesis. Hackeng WM, Brosens LA, Poruk KE, Noë M, Hosoda W, Poling JS, Rizzo A, Campbell-Thompson M, Atkinson MA, Konukiewitz B, Klöppel G, Heaphy CM, Meeker AK, Wood LD.
Hum Pathol. 2016 Jun 21. doi : 10.1016/j.humpath.2016.06 006
PMID: 27342911

 Loss of ATRX or DAXX expression and concomitant acquisition of the alternative lengthening of telomeres phenotype are late events in a small subset of MEN-1 syndrome pancreatic neuroendocrine tumors. de Wilde RF, Heaphy CM, Maitra A, Meeker AK, Edil BH, Wolfgang CL, Ellison TA, Schulick RD, Molenaar IQ, Valk GD, Vriens MR, Borel Rinkes IH, Offerhaus GJ, Hruban RH, Matsukuma KE. Mod Pathol. 2012 May 11. PMID: 22575867

 Marx SJ. Molecular genetics of multiple endocrine neoplasia types 1 and 2. Nat Rev Cancer. 2005 May;5(5):367-75. PMID: 15864278

 Agarwal SK, Lee Burns A, Sukhodolets KE, Kennedy PA, Obungu VH, Hickman AB, Mullendore ME, Whitten I, Skarulis MC, Simonds WF, Mateo C, Crabtree JS, Scacheri PC, Ji Y, Novotny EA, Garrett-Beal L, Ward JM, Libutti SK, Richard Alexander H, Cerrato A, Parisi MJ, Santa Anna-A S, Oliver B, Chandrasekharappa SC, Collins FS, Spiegel AM, Marx SJ. Molecular pathology of the MEN1 gene. Ann N Y Acad Sci. 2004 Apr;1014:189-98. PMID: 15153434

 Schussheim DH, Skarulis MC, Agarwal SK, Simonds WF, Burns AL, Spiegel AM, Marx SJ. Multiple endocrine neoplasia type 1: new clinical and basic findings. Trends Endocrinol Metab. 2001 May-Jun;12(4):173-8. PMID: 11295574