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RET Proto-Oncogene and Its Role in Multiple Endocrine Neoplasia and Medullary Thyroid Cancer

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Hormones and Cancer

Part of the book series: Hormones in Health and Disease ((HHD))

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Abstract

The RET proto-oncogene encodes a transmembrane protein receptor tyrosine kinase, the function of which is unknown at present. The inclusion of a discussion of this gene in a book on hormones and cancer is appropriate because of the recently described association of mutations in RET and the hereditary cancer syndromes multiple endocrine neoplasia (MEN) type 2A, MEN 2B, and familial non-MEN medullary thyroid cancer (FMTC). In these disorders, which are inherited in an autosomal dominant fashion, individuals inherit a predisposition to medullary thyroid cancer (MTC), a malignancy of neuroendocrine cells (C-cells) that reside in the para-follicular areas of the thyroid gland. This trait is expressed with virtually 100% penetrance. MEN 2A, MEN 2B, and FMTC are clinically distinct diseases. (Schimke, 1984, Farndon et al., 1986). MEN 2A and MEN 2B are syndromes characterized by neoplasms involving more than one endocrine tissue or cell type. FMTC, on the other hand, is a nonsyndromic Mendelian trait characterized by the development of MTC in the absence of any other recognizable abnormalities (Table 1). Through DNA marker linkage studies, the gene(s) responsible for MEN 2A, MEN 2B, and FMTC were mapped to the same region of chromosome 10 (Mathew et al., 1987a; Simpson et al., 1987; Norum et al., 1990a; Lairmore et al., 1992).

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Authors
  1. Paul Goodfellow
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  2. Jeffrey F. Moley
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Editors and Affiliations

  1. Department of Biochemistry and Molecular Biology, Louisiana State University Medical Center, 1901 Perdido Street, 70112-1393, New Orleans, LA, USA

    Wayne V. Vedeckis

  2. Stanley S. Scott Cancer Center, 1901 Perdido Street, 70112-1393, New Orleans, LA, USA

    Wayne V. Vedeckis

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© 1996 Birkhäuser Boston

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Goodfellow, P., Moley, J.F. (1996). RET Proto-Oncogene and Its Role in Multiple Endocrine Neoplasia and Medullary Thyroid Cancer. In: Vedeckis, W.V. (eds) Hormones and Cancer. Hormones in Health and Disease. Birkhäuser Boston. https://doi.org/10.1007/978-1-4612-4266-6_3

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  • DOI: https://doi.org/10.1007/978-1-4612-4266-6_3

  • Publisher Name: Birkhäuser Boston

  • Print ISBN: 978-1-4612-8715-5

  • Online ISBN: 978-1-4612-4266-6

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