Literature DB >> 16343738

Multiple endocrine neoplasia type 2 and the RET protooncogene: from bedside to bench to bedside.

Andreas Machens1, Henning Dralle.   

Abstract

Although the initial characterization of the various MEN-2 associated phenotypes (familial medullary thyroid cancer, multiple endocrine neoplasia 2A and 2B) evolved at the bedside, it was at the bench where the underlying RET (REarranged during Transfection) germline mutations were identified. Molecular information has revolutionized our understanding and continues to transform the clinical management of this fascinating endocrine tumor syndrome of neural crest derivation, which consists of medullary thyroid cancer, pheochromocytoma, and parathyroid hyperplasia/adenoma. DNA-based identification of RET carriers did not require comprehension of the gene, but was a prerequisite for clarifying gene function and devising biologic compounds blocking RET phosphorylation. With the continuing expansion of our knowledge about the underlying molecular mechanisms and our growing therapeutic abilities, multiple endocrine neoplasia type 2 is gradually returning home to the bedside, closing the loop from bedside to bench to bedside.

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Year:  2005        PMID: 16343738     DOI: 10.1016/j.mce.2005.10.028

Source DB:  PubMed          Journal:  Mol Cell Endocrinol        ISSN: 0303-7207            Impact factor:   4.102


  8 in total

Review 1.  Multiple endocrine neoplasia syndromes, children, Hirschsprung's disease and RET.

Authors:  S W Moore; M G Zaahl
Journal:  Pediatr Surg Int       Date:  2008-03-26       Impact factor: 1.827

2.  A family presenting with multiple endocrine neoplasia type 2B: A case report.

Authors:  Mahnaz Majidi; Vahid Haghpanah; Mahdi Hedayati; Patricia Khashayar; Mohammad Reza Mohajeri-Tehrani; Bagher Larijani
Journal:  J Med Case Rep       Date:  2011-12-20

3.  2012 European thyroid association guidelines for genetic testing and its clinical consequences in medullary thyroid cancer.

Authors:  R Elisei; M Alevizaki; B Conte-Devolx; K Frank-Raue; V Leite; G R Williams
Journal:  Eur Thyroid J       Date:  2012-12-19

Review 4.  Molecular genetics in primary hyperparathyroidism: the role of genetic tests in differential diagnosis, disease prevention strategy, and therapeutic planning. A 2017 update.

Authors:  Francesca Marini; Luisella Cianferotti; Francesca Giusti; Maria Luisa Brandi
Journal:  Clin Cases Miner Bone Metab       Date:  2017-05-30

Review 5.  Genetics of Cushing's Syndrome.

Authors:  Laura C Hernández-Ramírez; Constantine A Stratakis
Journal:  Endocrinol Metab Clin North Am       Date:  2018-06       Impact factor: 4.741

Review 6.  [Thyroid C cells and their pathology: Part 2: Medullary thyroid carcinoma].

Authors:  S Synoracki; S T Schmid; S Ting; K W Schmid
Journal:  Pathologe       Date:  2015-05       Impact factor: 1.011

Review 7.  Genotype-phenotype based surgical concept of hereditary medullary thyroid carcinoma.

Authors:  Andreas Machens; Henning Dralle
Journal:  World J Surg       Date:  2007-05       Impact factor: 3.352

Review 8.  A differential diagnosis of inherited endocrine tumors and their tumor counterparts.

Authors:  Sergio P A Toledo; Delmar M Lourenço; Rodrigo A Toledo
Journal:  Clinics (Sao Paulo)       Date:  2013-07       Impact factor: 2.365

  8 in total

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