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: 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)