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

First Author  Guo SS Year  2001
Journal  Mol Endocrinol Volume  15
Issue  10 Pages  1653-64
PubMed ID  11579199 Mgi Jnum  J:71967
Mgi Id  MGI:2151319 Doi  10.1210/mend.15.10.0717
Citation  Guo SS, et al. (2001) Molecular and genetic mechanisms of tumorigenesis in multiple endocrine neoplasia type-1. Mol Endocrinol 15(10):1653-64
abstractText  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 TGFbeta 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.
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