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Molecular pathology of the MEN1 gene.
Ann N Y Acad Sci. 2004 Apr; 1014:189-98.AN

Abstract

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.

Authors+Show Affiliations

National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

15153434

Citation

Agarwal, Sunita K., et al. "Molecular Pathology of the MEN1 Gene." Annals of the New York Academy of Sciences, vol. 1014, 2004, pp. 189-98.
Agarwal SK, Lee Burns A, Sukhodolets KE, et al. Molecular pathology of the MEN1 gene. Ann N Y Acad Sci. 2004;1014:189-98.
Agarwal, S. K., Lee Burns, A., Sukhodolets, K. E., Kennedy, P. A., Obungu, V. H., Hickman, A. B., Mullendore, M. E., Whitten, I., Skarulis, M. C., Simonds, W. F., Mateo, C., Crabtree, J. S., Scacheri, P. C., Ji, Y., Novotny, E. A., Garrett-Beal, L., Ward, J. M., Libutti, S. K., Richard Alexander, H., ... Marx, S. J. (2004). Molecular pathology of the MEN1 gene. Annals of the New York Academy of Sciences, 1014, 189-98.
Agarwal SK, et al. Molecular Pathology of the MEN1 Gene. Ann N Y Acad Sci. 2004;1014:189-98. PubMed PMID: 15153434.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Molecular pathology of the MEN1 gene. AU - Agarwal,Sunita K, AU - Lee Burns,A, AU - Sukhodolets,Karen E, AU - Kennedy,Patricia A, AU - Obungu,Victor H, AU - Hickman,Alison B, AU - Mullendore,Michael E, AU - Whitten,Ira, AU - Skarulis,Monica C, AU - Simonds,William F, AU - Mateo,Carmen, AU - Crabtree,Judy S, AU - Scacheri,Peter C, AU - Ji,Youngmi, AU - Novotny,Elizabeth A, AU - Garrett-Beal,Lisa, AU - Ward,Jerrold M, AU - Libutti,Steven K, AU - Richard Alexander,H, AU - Cerrato,Aniello, AU - Parisi,Michael J, AU - Santa Anna-A,Sonia, AU - Oliver,Brian, AU - Chandrasekharappa,Settara C, AU - Collins,Francis S, AU - Spiegel,Allen M, AU - Marx,Stephen J, PY - 2004/5/22/pubmed PY - 2004/6/16/medline PY - 2004/5/22/entrez SP - 189 EP - 98 JF - Annals of the New York Academy of Sciences JO - Ann N Y Acad Sci VL - 1014 N2 - 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. SN - 0077-8923 UR - https://www.unboundmedicine.com/medline/citation/15153434/Molecular_pathology_of_the_MEN1_gene_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0077-8923&date=2004&volume=1014&spage=189 DB - PRIME DP - Unbound Medicine ER -