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Heterozygous Men1 mutant mice develop a range of endocrine tumors mimicking multiple endocrine neoplasia type 1.
Mol Endocrinol. 2003 Sep; 17(9):1880-92.ME

Abstract

Multiple endocrine neoplasia type 1 (MEN1) is a hereditary syndrome characterized by the occurrence of multiple endocrine tumors of the parathyroid, pancreas, and anterior pituitary in patients. To study tumorigenesis related to the MEN1 syndrome, we have generated Men1 knockout mice using the gene targeting approach. Heterozygous Men1 mutant mice developed the same range of major endocrine tumors as is seen in MEN1 patients, affecting the parathyroid, pancreatic islets, pituitary and adrenal glands, as well as the thyroid, and exhibiting multistage tumor progression with metastatic potential. In particular, extrapancreatic gastrinoma, pancreatic glucagonoma, and mixed hormone-producing tumors in islets were observed. In addition, there was a high incidence of gonadal tumors of endocrine origin, i.e. Leydig cell tumors, and ovary sex-cord stromal cell tumors in heterozygous Men1 mutant mice. Hormonal disturbance, such as abnormal PTH and insulin levels, was also observed in these mice. These tumors were associated with loss of heterozygosity of the wild-type Men1 allele, suggesting that menin is involved in suppressing the development of these endocrine tumors. All of these features are reminiscent of MEN1 symptoms in humans and establish heterozygous Men1 mutant mice as a suitable model for this disease.

Authors+Show Affiliations

Laboratory of Genetics, Centre National de la Recherche Scientifique, Faculty of Medicine, University of Lyon, Lyon, France.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

12819299

Citation

Bertolino, Philippe, et al. "Heterozygous Men1 Mutant Mice Develop a Range of Endocrine Tumors Mimicking Multiple Endocrine Neoplasia Type 1." Molecular Endocrinology (Baltimore, Md.), vol. 17, no. 9, 2003, pp. 1880-92.
Bertolino P, Tong WM, Galendo D, et al. Heterozygous Men1 mutant mice develop a range of endocrine tumors mimicking multiple endocrine neoplasia type 1. Mol Endocrinol. 2003;17(9):1880-92.
Bertolino, P., Tong, W. M., Galendo, D., Wang, Z. Q., & Zhang, C. X. (2003). Heterozygous Men1 mutant mice develop a range of endocrine tumors mimicking multiple endocrine neoplasia type 1. Molecular Endocrinology (Baltimore, Md.), 17(9), 1880-92.
Bertolino P, et al. Heterozygous Men1 Mutant Mice Develop a Range of Endocrine Tumors Mimicking Multiple Endocrine Neoplasia Type 1. Mol Endocrinol. 2003;17(9):1880-92. PubMed PMID: 12819299.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Heterozygous Men1 mutant mice develop a range of endocrine tumors mimicking multiple endocrine neoplasia type 1. AU - Bertolino,Philippe, AU - Tong,Wei-Min, AU - Galendo,Dominique, AU - Wang,Zhao-Qi, AU - Zhang,Chang-Xian, Y1 - 2003/06/20/ PY - 2003/6/24/pubmed PY - 2004/5/12/medline PY - 2003/6/24/entrez SP - 1880 EP - 92 JF - Molecular endocrinology (Baltimore, Md.) JO - Mol Endocrinol VL - 17 IS - 9 N2 - Multiple endocrine neoplasia type 1 (MEN1) is a hereditary syndrome characterized by the occurrence of multiple endocrine tumors of the parathyroid, pancreas, and anterior pituitary in patients. To study tumorigenesis related to the MEN1 syndrome, we have generated Men1 knockout mice using the gene targeting approach. Heterozygous Men1 mutant mice developed the same range of major endocrine tumors as is seen in MEN1 patients, affecting the parathyroid, pancreatic islets, pituitary and adrenal glands, as well as the thyroid, and exhibiting multistage tumor progression with metastatic potential. In particular, extrapancreatic gastrinoma, pancreatic glucagonoma, and mixed hormone-producing tumors in islets were observed. In addition, there was a high incidence of gonadal tumors of endocrine origin, i.e. Leydig cell tumors, and ovary sex-cord stromal cell tumors in heterozygous Men1 mutant mice. Hormonal disturbance, such as abnormal PTH and insulin levels, was also observed in these mice. These tumors were associated with loss of heterozygosity of the wild-type Men1 allele, suggesting that menin is involved in suppressing the development of these endocrine tumors. All of these features are reminiscent of MEN1 symptoms in humans and establish heterozygous Men1 mutant mice as a suitable model for this disease. SN - 0888-8809 UR - https://www.unboundmedicine.com/medline/citation/12819299/Heterozygous_Men1_mutant_mice_develop_a_range_of_endocrine_tumors_mimicking_multiple_endocrine_neoplasia_type_1_ L2 - https://academic.oup.com/mend/article-lookup/doi/10.1210/me.2003-0154 DB - PRIME DP - Unbound Medicine ER -