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The Menin Gene

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Endocrine Neoplasia

Part of the book series: Cancer Treatment and Research ((CTAR,volume 153))

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Abstract

Multiple endocrine neoplasia type I (MEN-1) is an autosomal dominant syndrome featuring tumors of endocrine origin. Heterozygous germline mutations in the MEN-1 tumor suppressor gene predispose MEN-1 patients to tumor development, mainly in parathyroid, pancreatic islet cells, and the anterior pituitary gland. Since the MEN-1-encoded protein, menin, is ubiquitously expressed, the endocrine-specific nature in MEN-1 patients remains unexplained. This chapter provides an overview of the MEN-1 gene, including patterns of mutations identified since its discovery in 1997. Different menin-interacting protein partners and menin’s proposed molecular functions are also discussed. Lastly, various animal models of MEN-1 are described in detail. While analyses utilizing genetic, biochemical, and physiological techniques have led to a better understanding of menin’s mechanisms of action, much is yet to be elucidated about menin’s role in MEN-1 tumorigenesis.

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  1. Department of Surgery, Director, Montefiore-Einstein Center for Cancer Care., Bronx, NY, USA

    Steven K. Libutti (Professor of Surgery, Vice Chairman)

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  1. Hsin-Chieh Jennifer Shen
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Correspondence to Steven K. Libutti .

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  1. Feinberg School of Medicine, Northwestern University, E. Huron St. 201, Chicago, 60611, U.S.A.

    Cord Sturgeon

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Shen, HC.J., Libutti, S.K. (2010). The Menin Gene. In: Sturgeon, C. (eds) Endocrine Neoplasia. Cancer Treatment and Research, vol 153. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-0857-5_15

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