PMID- 11579199 OWN - NLM STAT- MEDLINE DCOM- 20020221 LR - 20051116 IS - 0888-8809 (Print) IS - 0888-8809 (Linking) VI - 15 IP - 10 DP - 2001 Oct TI - Molecular and genetic mechanisms of tumorigenesis in multiple endocrine neoplasia type-1. PG - 1653-64 AB - 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. FAU - Guo, S S AU - Guo SS AD - Department of Surgery, West Los Angeles Veterans Affairs Medical Center and the University of California Los Angeles School of Medicine, Los Angeles, California 90073, USA. FAU - Sawicki, M P AU - Sawicki MP LA - eng PT - Journal Article PT - Review PL - United States TA - Mol Endocrinol JT - Molecular endocrinology (Baltimore, Md.) JID - 8801431 RN - 0 (MEN1 protein, human) RN - 0 (Neoplasm Proteins) RN - 0 (Proto-Oncogene Proteins) SB - IM MH - Amino Acid Sequence MH - Animals MH - Gene Expression MH - Humans MH - Molecular Sequence Data MH - Multiple Endocrine Neoplasia Type 1/diagnosis/*genetics MH - Mutation MH - Neoplasm Proteins/chemistry/genetics MH - *Proto-Oncogene Proteins MH - Sequence Alignment RF - 131 EDAT- 2001/10/02 10:00 MHDA- 2002/02/22 10:01 CRDT- 2001/10/02 10:00 PHST- 2001/10/02 10:00 [pubmed] PHST- 2002/02/22 10:01 [medline] PHST- 2001/10/02 10:00 [entrez] AID - 10.1210/mend.15.10.0717 [doi] PST - ppublish SO - Mol Endocrinol. 2001 Oct;15(10):1653-64. doi: 10.1210/mend.15.10.0717.