Familial multiple endocrine neoplasia type 1 Mutation of a tumor suppressor gene

Marx, Stephen J.

doi:10.1016/1043-2760(89)90007-6

Cited by 5 publications

(2 citation statements)

References 33 publications

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“…According to the two-hit model of tumorigenesis, both copies of a gene situated on opposite alleles must be inactivated, such as by deletion, rearrangement, or silencing through methylation, to confer selective growth advantage to a precursor cell that may subsequently proliferate in a clonal neoplastic fashion (277). MEN-1 (menin) gene.…”

Section: Tumor Suppressor Gene (Tsg) Inactivationmentioning

confidence: 99%

The Cytogenesis and Pathogenesis of Pituitary Adenomas*

1998

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Section: Tumor Suppressor Gene (Tsg) Inactivationmentioning

confidence: 99%

The Cytogenesis and Pathogenesis of Pituitary Adenomas*

1998

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“…Multiple Endocrine Neoplasia type-1 (MEN-1; menin) Gene According to the two-hit model of tumorigenesis, both copies of a gene situated on opposite alleles have to be inactivated, such as by deletion, rearrangement, or silencing through methylation, to confer selective growth advantage to a precursor cell which may subsequently proliferate in a clonal neoplastic fashion. 82 Such genes which require homozygous inactivation of both copies are termed TSGs, antioncogenes or recessive oncogenes. Current examples of TSGs include the retinoblastoma (Rb) gene , p53, and the colorectal carcinoma gene deleted in colon cancer (DCC).…”

Section: Loss Of Function Tumor Suppressor Signaling Proteinsmentioning

confidence: 99%

The Pathophysiology of Pituitary Tumors

Ezzat¹,

Sylvia²

2002

Seminars in Neurosurgery

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Numerous factors have been shown to govern pituitary cell proliferation; these various hypophysiotropic hormones and growth factors (GFs) likely play a role as promoters of tumor cell growth in genetically transformed cells. The clonal composition of pituitary adenomas attests to the molecular basis of pituitary tumorigenesis; however, the oncogenes and tumor suppressor genes that are implicated in the transformation events for the vast majority of pituitary tumors remain unknown. Mutations that have been identified in other human malignancies are restricted to a very small subset of pituitary neoplasms implicating novel genetic and/or epigenetic alterations. This review details some of the currently known alterations of GFs and their receptors that have been implicated in pituitary tumorigenesis. Some of the epigenetic changes noted in nuclear components that govern cell cycle control are also reviewed. The emerging knowledge from these studies is shedding new light not only on the pathogenesis of pituitary tumors but on novel mechanisms in the broader context of human neoplasia.Objectives: On completion of this article, the reader should understand the mechanisms involved in the development of pituitary tumors and the differences between experimental models and the primary human pituitary adenomas.

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Journal of Bone and Mineral Research

Marx

1991

Journal of Bone and Mineral Research

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Primary hyperparathyroidism is caused by defects in the parathyroid gland. Investigations have implicated three interesting genes whose mutation can cause primary hyperparathyroidism. Familial hypocalciuric hypercalcemia is believed to be an atypical form of primary hyperparathyroidism with an inherited defect in calcium recognition expressed not only in all parathyroid chief cells (thus a polyclonal defect) but in some renal tubular cells as well. In typical primary hyperparathyroidism a monoclonal parathyroid tumor is usually the central cause. Either of two apparently different genes on the long arm of chromosome 11 has been implicated in development of a parathyroid tumor clone. One gene (D11S287) was shown to have undergone a rearrangement with the parathyroid hormone gene on the short arm of the same chromosome (pericentromeric inversion) in a small fraction of tumors; the D11S287 locus may encode a growth stimulator. Another gene, the locus for familial multiple endocrine neoplasia type 1 (FEMEN1), is likely to encode a growth inhibitor. Inactivation of this gene or another nearby gene by somatic mutation has been indirectly implicated in one-quarter of sporadic parathyroid adenomas and in more than half of parathyroid tumors in FMEN1. In conclusion, studies have suggested three different mechanisms for parathyroid gland dysfunction in primary hyperparathyroidism: (1) a defect in calcium recognition, (2) a monoclonal tumor from overexpression of a growth stimulator, or (3) a monoclonal tumor from inactivation of a growth inhibitor.

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Familial multiple endocrine neoplasia type 1 Mutation of a tumor suppressor gene

Cited by 5 publications

References 33 publications

The Cytogenesis and Pathogenesis of Pituitary Adenomas*

The Cytogenesis and Pathogenesis of Pituitary Adenomas*

The Pathophysiology of Pituitary Tumors

Journal of Bone and Mineral Research

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