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Multiple endocrine neoplasia type 1 (MEN-1) is an autosomal dominant disorder characterized by the combined occurrence of tumours of the parathyroids, pancreatic islet cells and anterior pituitary. Other MEN-1-associated tumours include angiofibromas, collagenomas, lipomas, carcinoids and adrenocortical tumours. The MEN-1 gene, which represents a putative tumour suppressor gene, was identified in 1997, and over 340 mutations have been reported in MEN-1 families, patients with non-familial MEN-1, families with isolated primary hyperparathyroidism and those with sporadic non-MEN-1 endocrine tumours. The mutations are scattered throughout the nine exons that encode a 610 amino acid nuclear protein (MENIN), which interacts with the transcriptional factor JunD. These recent developments have made it possible to consider genetic screening for this inherited disorder.

Original publication

DOI

10.1053/beem.2001.0135

Type

Journal article

Journal

Best Practice and Research: Clinical Endocrinology and Metabolism

Publication Date

20/08/2001

Volume

15

Pages

189 - 212