Molecular and genetic mechanisms of tumorigenesis in multiple endocrine neoplasia type-1

Mol Endocrinol. 2001 Oct;15(10):1653-64. doi: 10.1210/mend.15.10.0717.


Multiple endocrine neoplasia type 1 (MEN1) is a rare but informative syndrome for endocrine tumorigenesis. Since its isolation, several groups have begun to determine the role of menin, the protein product of MEN1, in sporadic endocrine tumors as well as tumors of the MEN1 syndrome. Mutations of menin have been reported in more than 400 families and tumors, most of which are truncating mutations, thus supporting the function of menin as a tumor suppressor. The exact function of menin is unknown, but overexpression of menin inhibits proliferation of Ras-transformed NIH3T3 cells. Since menin interacts with proteins from both the TGF beta and AP-1 signaling pathways, perhaps its tumor suppressor function is related to these key cell growth pathways. In this review we will discuss the various clinical manifestations of MEN1 syndrome, potential mechanisms of MEN1 tumorigenesis, and mutations associated with MEN and sporadic endocrine tumors.

Publication types

  • Review

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Gene Expression
  • Humans
  • Molecular Sequence Data
  • Multiple Endocrine Neoplasia Type 1 / diagnosis
  • Multiple Endocrine Neoplasia Type 1 / genetics*
  • Mutation
  • Neoplasm Proteins / chemistry
  • Neoplasm Proteins / genetics
  • Proto-Oncogene Proteins*
  • Sequence Alignment


  • MEN1 protein, human
  • Neoplasm Proteins
  • Proto-Oncogene Proteins