Multiple Endocrine Neoplasia type 1.
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.
Piecha
Grzegorz
G
Department of Nephrology, Endocrinology and Metabolic Diseases, Medical University of Silesia, Katowice, ul. Francuska 20/24, 40-027 Katowice, Poland.
Chudek
Jerzy
J
Wiecek
Andrzej
A
D015408:Gastrinoma / Q000175:diagnosis / Q000235:genetics; D015870:Gene Expression; D006801:Humans; D006961:Hyperparathyroidism / Q000175:diagnosis / Q000235:genetics; D007340:Insulinoma / Q000175:diagnosis / Q000235:genetics; D018761:Multiple Endocrine Neoplasia Type 1 / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D009154:Mutation; D015175:Prolactinoma / Q000175:diagnosis / Q000235:genetics; D011518:Proto-Oncogene Proteins / Q000235:genetics / Q000502:physiology*
C105373:MEN1 protein, human; D011518:Proto-Oncogene Proteins