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

  • Paul Goodfellow
  • Jeffrey F. Moley
Part of the Hormones in Health and Disease book series (HHD)

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).

Keywords

Multiple Endocrine Neoplasia Type Multiple Endocrine Neoplasia Medullary Thyroid Cancer Familial Medullary Thyroid Carcinoma Plasma Calcitonin Level 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Birkhäuser Boston 1996

Authors and Affiliations

  • Paul Goodfellow
  • Jeffrey F. Moley

There are no affiliations available

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