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A mouse model of multiple endocrine neoplasia, type 1, develops multiple endocrine tumors.
Proc Natl Acad Sci U S A. 2001 Jan 30; 98(3):1118-23.PN

Abstract

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant cancer syndrome, characterized primarily by multiple tumors in the parathyroid glands, endocrine pancreas, and anterior pituitary. Other tumors, including gastrinoma, carcinoid, adrenal cortical tumors, angiofibroma, collagenoma, and lipoma, also occur in some patients. Individuals with MEN1 almost always have loss-of-function mutations in the MEN1 gene on chromosome 11, and endocrine tumors arising in these patients usually show somatic loss of the remaining wild-type allele. To examine the role of MEN1 in tumor formation, a mouse model was generated through homologous recombination of the mouse homolog Men1. Homozygous mice die in utero at embryonic days 11.5-12.5, whereas heterozygous mice develop features remarkably similar to those of the human disorder. As early as 9 months, pancreatic islets show a range of lesions from hyperplasia to insulin-producing islet cell tumors, and parathyroid adenomas are also frequently observed. Larger, more numerous tumors involving pancreatic islets, parathyroids, thyroid, adrenal cortex, and pituitary are seen by 16 months. All of the tumors tested to date show loss of the wild-type Men1 allele, further supporting its role as a tumor suppressor gene.

Authors+Show Affiliations

National Human Genome Research Institute, National Cancer Institute, and National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 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 available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

11158604

Citation

Crabtree, J S., et al. "A Mouse Model of Multiple Endocrine Neoplasia, Type 1, Develops Multiple Endocrine Tumors." Proceedings of the National Academy of Sciences of the United States of America, vol. 98, no. 3, 2001, pp. 1118-23.
Crabtree JS, Scacheri PC, Ward JM, et al. A mouse model of multiple endocrine neoplasia, type 1, develops multiple endocrine tumors. Proc Natl Acad Sci U S A. 2001;98(3):1118-23.
Crabtree, J. S., Scacheri, P. C., Ward, J. M., Garrett-Beal, L., Emmert-Buck, M. R., Edgemon, K. A., Lorang, D., Libutti, S. K., Chandrasekharappa, S. C., Marx, S. J., Spiegel, A. M., & Collins, F. S. (2001). A mouse model of multiple endocrine neoplasia, type 1, develops multiple endocrine tumors. Proceedings of the National Academy of Sciences of the United States of America, 98(3), 1118-23.
Crabtree JS, et al. A Mouse Model of Multiple Endocrine Neoplasia, Type 1, Develops Multiple Endocrine Tumors. Proc Natl Acad Sci U S A. 2001 Jan 30;98(3):1118-23. PubMed PMID: 11158604.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A mouse model of multiple endocrine neoplasia, type 1, develops multiple endocrine tumors. AU - Crabtree,J S, AU - Scacheri,P C, AU - Ward,J M, AU - Garrett-Beal,L, AU - Emmert-Buck,M R, AU - Edgemon,K A, AU - Lorang,D, AU - Libutti,S K, AU - Chandrasekharappa,S C, AU - Marx,S J, AU - Spiegel,A M, AU - Collins,F S, PY - 2001/2/7/pubmed PY - 2001/4/17/medline PY - 2001/2/7/entrez SP - 1118 EP - 23 JF - Proceedings of the National Academy of Sciences of the United States of America JO - Proc Natl Acad Sci U S A VL - 98 IS - 3 N2 - Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant cancer syndrome, characterized primarily by multiple tumors in the parathyroid glands, endocrine pancreas, and anterior pituitary. Other tumors, including gastrinoma, carcinoid, adrenal cortical tumors, angiofibroma, collagenoma, and lipoma, also occur in some patients. Individuals with MEN1 almost always have loss-of-function mutations in the MEN1 gene on chromosome 11, and endocrine tumors arising in these patients usually show somatic loss of the remaining wild-type allele. To examine the role of MEN1 in tumor formation, a mouse model was generated through homologous recombination of the mouse homolog Men1. Homozygous mice die in utero at embryonic days 11.5-12.5, whereas heterozygous mice develop features remarkably similar to those of the human disorder. As early as 9 months, pancreatic islets show a range of lesions from hyperplasia to insulin-producing islet cell tumors, and parathyroid adenomas are also frequently observed. Larger, more numerous tumors involving pancreatic islets, parathyroids, thyroid, adrenal cortex, and pituitary are seen by 16 months. All of the tumors tested to date show loss of the wild-type Men1 allele, further supporting its role as a tumor suppressor gene. SN - 0027-8424 UR - https://www.unboundmedicine.com/medline/citation/11158604/A_mouse_model_of_multiple_endocrine_neoplasia_type_1_develops_multiple_endocrine_tumors_ L2 - http://www.pnas.org/cgi/pmidlookup?view=long&pmid=11158604 DB - PRIME DP - Unbound Medicine ER -