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Animal models of multiple endocrine neoplasia.
Mol Cell Endocrinol. 2016 Feb 05; 421:49-59.MC

Abstract

Multiple endocrine neoplasia (MEN) syndromes are autosomal dominant diseases with high penetrance characterized by proliferative lesions (usually hyperplasia or adenoma) arising in at least two endocrine tissues. Four different MEN syndromes have been so far identified: MEN type 1 (MEN1), MEN2A (also referred to as MEN2), MEN2B (or MEN3) and MEN4, which have slightly varying tumor spectra and are caused by mutations in different genes. MEN1 associates with loss-of-function mutations in the MEN1 gene encoding the tumor suppressor menin. The MEN2A and MEN2B syndromes are due to activating mutations in the proto-oncogene RET (Rearranged in Transfection) and are characterized by different phenotypic features of the affected patients. MEN4 was the most recent addition to the family of the MEN syndromes. It was discovered less than 10 years ago thanks to studies of a rat strain that spontaneously develops multiple endocrine tumors (named MENX). These studies identified an inactivating mutation in the Cdkn1b gene, encoding the putative tumor suppressor p27, as the causative mutation of the rat syndrome. Subsequently, germline mutations in the human ortholog CDKN1B were also found in a subset of patients with a MEN-like phenotype and this led to the identification of MEN4. Small animal models have been instrumental in understanding important biochemical, physiological and pathological processes of cancer onset and spread in intact living organisms. Moreover, they have provided us with insight into gene function(s) and molecular mechanisms of disease progression. We here review the currently available animal models of MEN syndromes and their impact on the elucidation of the pathophysiology of these diseases, with a special focus on the rat MENX syndrome that we have been characterizing.

Authors+Show Affiliations

Institute of Pathology, Helmholtz Zentrum München-German Research Center for Environmental Health, Ingolstaedter Landstrasse 1, 85764 Neuherberg, Germany.Institute of Pathology, Helmholtz Zentrum München-German Research Center for Environmental Health, Ingolstaedter Landstrasse 1, 85764 Neuherberg, Germany. Electronic address: natalia.pellegata@helmholtz-muenchen.de.

Pub Type(s)

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

Language

eng

PubMed ID

26184857

Citation

Wiedemann, Tobias, and Natalia S. Pellegata. "Animal Models of Multiple Endocrine Neoplasia." Molecular and Cellular Endocrinology, vol. 421, 2016, pp. 49-59.
Wiedemann T, Pellegata NS. Animal models of multiple endocrine neoplasia. Mol Cell Endocrinol. 2016;421:49-59.
Wiedemann, T., & Pellegata, N. S. (2016). Animal models of multiple endocrine neoplasia. Molecular and Cellular Endocrinology, 421, 49-59. https://doi.org/10.1016/j.mce.2015.07.004
Wiedemann T, Pellegata NS. Animal Models of Multiple Endocrine Neoplasia. Mol Cell Endocrinol. 2016 Feb 5;421:49-59. PubMed PMID: 26184857.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Animal models of multiple endocrine neoplasia. AU - Wiedemann,Tobias, AU - Pellegata,Natalia S, Y1 - 2015/07/13/ PY - 2015/05/07/received PY - 2015/06/23/revised PY - 2015/07/03/accepted PY - 2015/7/18/entrez PY - 2015/7/18/pubmed PY - 2016/10/13/medline KW - Endocrine neoplasia KW - Endocrine tumors KW - Menin KW - Multiple endocrine neoplasia syndromes KW - Ret KW - p27 SP - 49 EP - 59 JF - Molecular and cellular endocrinology JO - Mol Cell Endocrinol VL - 421 N2 - Multiple endocrine neoplasia (MEN) syndromes are autosomal dominant diseases with high penetrance characterized by proliferative lesions (usually hyperplasia or adenoma) arising in at least two endocrine tissues. Four different MEN syndromes have been so far identified: MEN type 1 (MEN1), MEN2A (also referred to as MEN2), MEN2B (or MEN3) and MEN4, which have slightly varying tumor spectra and are caused by mutations in different genes. MEN1 associates with loss-of-function mutations in the MEN1 gene encoding the tumor suppressor menin. The MEN2A and MEN2B syndromes are due to activating mutations in the proto-oncogene RET (Rearranged in Transfection) and are characterized by different phenotypic features of the affected patients. MEN4 was the most recent addition to the family of the MEN syndromes. It was discovered less than 10 years ago thanks to studies of a rat strain that spontaneously develops multiple endocrine tumors (named MENX). These studies identified an inactivating mutation in the Cdkn1b gene, encoding the putative tumor suppressor p27, as the causative mutation of the rat syndrome. Subsequently, germline mutations in the human ortholog CDKN1B were also found in a subset of patients with a MEN-like phenotype and this led to the identification of MEN4. Small animal models have been instrumental in understanding important biochemical, physiological and pathological processes of cancer onset and spread in intact living organisms. Moreover, they have provided us with insight into gene function(s) and molecular mechanisms of disease progression. We here review the currently available animal models of MEN syndromes and their impact on the elucidation of the pathophysiology of these diseases, with a special focus on the rat MENX syndrome that we have been characterizing. SN - 1872-8057 UR - https://www.unboundmedicine.com/medline/citation/26184857/Animal_models_of_multiple_endocrine_neoplasia_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0303-7207(15)30013-7 DB - PRIME DP - Unbound Medicine ER -