PMID- 15153434
OWN - NLM
STAT- MEDLINE
DCOM- 20040615
LR  - 20220331
IS  - 0077-8923 (Print)
IS  - 0077-8923 (Linking)
VI  - 1014
DP  - 2004 Apr
TI  - Molecular pathology of the MEN1 gene.
PG  - 189-98
AB  - Multiple endocrine neoplasia type 1 (MEN1), among all syndromes, causes tumors in 
      the highest number of tissue types. Most of the tumors are hormone producing 
      (e.g., parathyroid, enteropancreatic endocrine, anterior pituitary) but some are 
      not (e.g., angiofibroma). MEN1 tumors are multiple for organ type, for regions of 
      a discontinuous organ, and for subregions of a continuous organ. Cancer 
      contributes to late mortality; there is no effective prevention or cure for MEN1 
      cancers. Morbidities are more frequent from benign than malignant tumor, and both 
      are indicators for screening. Onset age is usually earlier in a tumor type of 
      MEN1 than of nonhereditary cases. Broad trends contrast with those in 
      nonneoplastic excess of hormones (e.g., persistent hyperinsulinemic hypoglycemia 
      of infancy). Most germline or somatic mutations in the MEN1 gene predict 
      truncation or absence of encoded menin. Similarly, 11q13 loss of heterozygosity 
      in tumors predicts inactivation of the other MEN1 copy. MEN1 somatic mutation is 
      prevalent in nonhereditary, MEN1-like tumor types. Compiled germline and somatic 
      mutations show almost no genotype/phenotype relation. Normal menin is 67 kDa, 
      widespread, and mainly nuclear. It may partner with junD, NF-kB, PEM, SMAD3, 
      RPA2, FANCD2, NM23beta, nonmuscle myosin heavy chain II-A, GFAP, and/or vimentin. 
      These partners have not clarified menin's pathways in normal or tumor tissues. 
      Animal models have opened approaches to menin pathways. Local overexpression of 
      menin in Drosophila reveals its interaction with the jun-kinase pathway. The 
      Men1+/- mouse has robust MEN1; its most important difference from human MEN1 is 
      marked hyperplasia of pancreatic islets, a tumor precursor stage.
FAU - Agarwal, Sunita K
AU  - Agarwal SK
AD  - National Institute of Diabetes and Digestive and Kidney Diseases, National 
      Institutes of Health, Bethesda, Maryland 20892, USA.
FAU - Lee Burns, A
AU  - Lee Burns A
FAU - Sukhodolets, Karen E
AU  - Sukhodolets KE
FAU - Kennedy, Patricia A
AU  - Kennedy PA
FAU - Obungu, Victor H
AU  - Obungu VH
FAU - Hickman, Alison B
AU  - Hickman AB
FAU - Mullendore, Michael E
AU  - Mullendore ME
FAU - Whitten, Ira
AU  - Whitten I
FAU - Skarulis, Monica C
AU  - Skarulis MC
FAU - Simonds, William F
AU  - Simonds WF
FAU - Mateo, Carmen
AU  - Mateo C
FAU - Crabtree, Judy S
AU  - Crabtree JS
FAU - Scacheri, Peter C
AU  - Scacheri PC
FAU - Ji, Youngmi
AU  - Ji Y
FAU - Novotny, Elizabeth A
AU  - Novotny EA
FAU - Garrett-Beal, Lisa
AU  - Garrett-Beal L
FAU - Ward, Jerrold M
AU  - Ward JM
FAU - Libutti, Steven K
AU  - Libutti SK
FAU - Richard Alexander, H
AU  - Richard Alexander H
FAU - Cerrato, Aniello
AU  - Cerrato A
FAU - Parisi, Michael J
AU  - Parisi MJ
FAU - Santa Anna-A, Sonia
AU  - Santa Anna-A S
FAU - Oliver, Brian
AU  - Oliver B
FAU - Chandrasekharappa, Settara C
AU  - Chandrasekharappa SC
FAU - Collins, Francis S
AU  - Collins FS
FAU - Spiegel, Allen M
AU  - Spiegel AM
FAU - Marx, Stephen J
AU  - Marx SJ
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Ann N Y Acad Sci
JT  - Annals of the New York Academy of Sciences
JID - 7506858
RN  - 0 (MEN1 protein, human)
RN  - 0 (Proto-Oncogene Proteins)
SB  - IM
MH  - Animals
MH  - *Gene Expression Regulation, Neoplastic
MH  - Humans
MH  - Multiple Endocrine Neoplasia/*genetics/pathology/*physiopathology
MH  - Proto-Oncogene Proteins/*genetics
RF  - 38
EDAT- 2004/05/22 05:00
MHDA- 2004/06/16 05:00
CRDT- 2004/05/22 05:00
PHST- 2004/05/22 05:00 [pubmed]
PHST- 2004/06/16 05:00 [medline]
PHST- 2004/05/22 05:00 [entrez]
AID - 10.1196/annals.1294.020 [doi]
PST - ppublish
SO  - Ann N Y Acad Sci. 2004 Apr;1014:189-98. doi: 10.1196/annals.1294.020.