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Multiple endocrine neoplasia Type 1 (MEN1) is a rare hereditary tumor syndrome predisposing to tumor development in several endocrine organs. Its major manifestations include hyperparathyroidism, tumors of endocrine pancreas and pituitary. Beside these three, several other endocrine (adrenocortical, foregut carcinoid) and nonendocrine (lipoma, angiofibroma, collagenoma, ependymoma, meningioma) tumors...
Multiple Endocrine Neoplasia Type 1 corresponds to a monogenic predisposition syndrome inherited as a dominant trait that affects a variety of endocrine tissues, in particular parathyroids, endocrine pancreas and anterior pituitary. It is caused by mutations in the MEN1 tumor suppressor gene that inactivate menin, the MEN1 encoded protein. Menin is involved in cell cycle control and apoptosis through...
The cloning of the MEN1 gene in 1997 led to the characterization of menin, the protein behind the multiple endocrine neoplasia Type 1 syndrome. Menin, a novel nuclear protein with no homologyto other gene products, is expressed ubiquitously. MEN1 missense mutations are dispersed along the coding region of the gene but are more common in the most conserved regions. Likewise, domains of protein interaction...
Since its discovery as a novel protein some 10 years ago, many cellular functions of menin have been identified. However, which ones of these relate specifically to menin’s role as a tumor suppressor and which ones not remains unclear. Menin is predominantly nuclear and acts as a scaffold protein to regulate gene transcription by coordinating chromatin remodeling. It is implicated in both histone...
In the hematopoietic system, menin was found to interact with MLL, a large protein encoded by the mixed linage leukemia gene that acts as a histone H3 methyltransferase. The MLL gene is a recurrent target for translocations in both acute myeloid and acute lymphoid leukemias. MLL gene rearrangements involve a variety of translocation partners, giving rise to MLL fusion proteins whose transforming ability...
Menin function is related to transcriptional regulation and cell cycle control and it physicallyand functionally interacts with osteotropic transcription factors, such as Smad1/5, Smad3, Runx2 and JunD. Menin promotes the commitment of pluripotent mesenchymal stem cells to the osteoblast lineage, mediated by interactions between menin and the BMP signaling molecules, Smad1/S, or Runx2. On the other...
Pituitary adenomas are common monoclonal neoplasms accounting for approximately one-fifth of primary intracranial tumors. Prolactin-secreting pituitary adenomas (prolactinomas) are the most common form of pituitary tumors in humans. They are associated with excessive release of the hormone prolactin and increased tumor growth, giving rise to severe endocrine disorders and serious clinical concerns...
Primary hyperparathyroidism is a common disorder that involves the pathological enlargement of one or more parathyroid glands resulting in excessive production of parathyroid hormone (PTH). The exact pathogenesis of this disease remains to be fully understood. In recent years interest has focussed on the interaction between menin protein and the transforming growth factor (TGF)-β/Smad signalling pathway...
The menin protein encoded by the MEN1 tumor suppressor gene is ubiquitously expressed and highly conserved evolutionarily. The combination of findings from current in vitro and in vivo studies has not yielded a comprehensive understanding of the mechanisms of menin’s tumor suppressor activity or the specific role for menin in endocrine tumorigenesis, although its diverse interactions suggest possible...
Most of the adrenal tumors are benign adrenocortical adenoma (AA)and pheochromocytomas (Pheo) originatingfrom the adrenal medulla, but rarely malignant adrenocortical carcinomas (ACC) can be also found. Adrenal tumors causinghormonal overproduction such as aldosterone-producing and cortisol-producingtumors are also rare, whereas nonhyperfunctioning adenomas occur more frequently.1 During the last...
To investigate the physiological role of menin, the protein product of the MEN1 gene, several groups have utilized gene targeting strategies to delete one or both copies of the mouse homolog Men1. Mice that arehomozygous null for Men1 die duringembryogenesis. Heterozygous Men1 mice are viable and develop many of the same types of tumors as humans with MEN1. In addition to conventional knockouts of...
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