Abstract
The co-occurrence of parathyroid hyperplasia with pancreatic endocrine tumours and/or pituitary adenoma is classified as Multiple Endocrine Neoplasia type 1 (MEN-1) and is caused by a germ-line mutation in MEN-1 gene encoding a tumour suppressor protein, menin. This review presents clinical expressions, diagnosis and management of the MEN-1 syndrome. Properties and mechanisms of menin functions are also reviewed.
MeSH terms
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Gastrinoma / diagnosis
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Gastrinoma / genetics
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Gene Expression
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Humans
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Hyperparathyroidism / diagnosis
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Hyperparathyroidism / genetics
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Insulinoma / diagnosis
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Insulinoma / genetics
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Multiple Endocrine Neoplasia Type 1 / diagnosis
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Multiple Endocrine Neoplasia Type 1 / genetics*
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Multiple Endocrine Neoplasia Type 1 / therapy
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Mutation
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Prolactinoma / diagnosis
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Prolactinoma / genetics
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Proto-Oncogene Proteins / genetics
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Proto-Oncogene Proteins / physiology*
Substances
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MEN1 protein, human
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Proto-Oncogene Proteins