title: Tissue selectivity in multiple endocrine neoplasia type 1-associated tumorigenesis.
abstract: The phenotype of the multiple endocrine neoplasia type 1 (MEN1) syndrome cannot be explained solely by the expression pattern of the predisposing gene MEN1 and its encoded protein, menin. This review addresses putative factors determining MEN1-associated tissue-selective tumorigenesis. Menin's interaction with mixed-lineage leukemia protein-containing histone methyl transferase (MLL-HMT) complex mediates tissue-selective tumor-suppressing and tumor-promoting effects of menin, and as such could be decisive for the predisposition of individual tissues to MEN1-associated tumorigenesis. In tissues in which menin acts as a tumor suppressor, tumorigenesis could depend on the inability of such tissues to adequately compensate for MEN1 gene loss, whereas the variable clinical presentation of MEN1 in individual patients could be a reflection of additional epigenetic factors and/or modifier genes. Further research on this topic may facilitate development of novel therapeutic strategies that could prevent or delay the onset of MEN1-associated tumorigenesis.
authors: Department of Clinical Sciences of Companion Animals, Faculty of Veterinary Medicine, Utrecht University, Utrecht, The Netherlands.
authors: Ana
authors: A
authors: Gracanin
authors: Koen M A
authors: KM
authors: Dreijerink
authors: Rob B
authors: RB
authors: van der Luijt
authors: Cornelis J M
authors: CJ
authors: Lips
authors: Jo W M
authors: JW
authors: Höppener
mesh_terms: D000818:Animals; D002471:Cell Transformation, Neoplastic / Q000235:genetics*; D017353:Gene Deletion; D006801:Humans; D008954:Models, Biological; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications / Q000235:genetics; D009369:Neoplasms / Q000209:etiology / Q000235:genetics*; D009928:Organ Specificity / Q000235:genetics; D011518:Proto-Oncogene Proteins / Q000235:genetics* / Q000502:physiology
chemical_list: C105373:MEN1 protein, human; D011518:Proto-Oncogene Proteins