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 | Ana Gracanin, Koen M A Dreijerink, Rob B van der Luijt, Cornelis J M Lips, Jo W M Höppener |
Journal | Cancer research
(Cancer Res)
Vol. 69
Issue 16
Pg. 6371-4
(Aug 15 2009)
ISSN: 1538-7445 [Electronic] United States |
PMID | 19654304
(Publication Type: Journal Article, Review)
|
Chemical References |
- MEN1 protein, human
- Proto-Oncogene Proteins
|
Topics |
- Animals
- Cell Transformation, Neoplastic
(genetics)
- Gene Deletion
- Humans
- Models, Biological
- Multiple Endocrine Neoplasia Type 1
(complications, genetics)
- Neoplasms
(etiology, genetics)
- Organ Specificity
(genetics)
- Proto-Oncogene Proteins
(genetics, physiology)
|