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Probl Endokrinol (Mosk) ; 66(2): 4-12, 2020 08 30.
Article in Russian | MEDLINE | ID: mdl-33351343

ABSTRACT

Changes in the expression of non-coding ribonucleic acids (ncRNAs) take part in the formation of various tumors. Multiple endocrine neoplasia syndrome type 1 (MEN1) is a rare autosomal dominant disease caused by mutations of the MEN1 gene encoding the menin protein. This syndrome is characterized by the occurrence of parathyroid tumors, gastroenteropancreatic neuroendocrine tumors, pituitary adenomas, as well as other endocrine and non-endocrine tumors. The pathogenesis of MEN-1 associated tumors due to MEN1 mutations remains unclear. In the absence of mutations of the MEN1 gene in patients with phenotypically similar features, this condition is regarded as a phenocopy of this syndrome. The cause of the combination of several MEN-1-related tumors in these patients remains unknown. The possible cause is that changes in the expression of ncRNAs affect the regulation of signaling pathways in which menin participates and may contribute to the development of MEN-1-related tumors. The identification of even a small number of agents interacting with menin makes a significant contribution to the improvement of knowledge about its pathophysiological influence and ways of developing tumors within the MEN-1 syndrome and its phenocopies.


Subject(s)
Multiple Endocrine Neoplasia Type 1 , Neuroendocrine Tumors , Pancreatic Neoplasms , Parathyroid Neoplasms , Pituitary Neoplasms , Humans , Multiple Endocrine Neoplasia Type 1/genetics , Pancreatic Neoplasms/genetics , Parathyroid Neoplasms/genetics , Pituitary Neoplasms/genetics
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