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Germline RET Leu56Met Variant Is Likely Not Causative of Multiple Endocrine Neoplasia Type 2

Title: Germline RET Leu56Met Variant Is Likely Not Causative of Multiple Endocrine Neoplasia Type 2
Authors: Hansen, Anna Reimer; Borgwardt, Line; Rasmussen, Åse Krogh; Godballe, Christian; Poulsen, Morten Møller; Vieira, Filipe G.; Mathiesen, Jes Sloth; Rossing, Maria
Source: Hansen, A R, Borgwardt, L, Rasmussen, Å K, Godballe, C, Poulsen, M M, Vieira, F G, Mathiesen, J S & Rossing, M 2021, 'Germline RET Leu56Met Variant Is Likely Not Causative of Multiple Endocrine Neoplasia Type 2', Frontiers in Endocrinology, vol. 12, 764512. https://doi.org/10.3389/fendo.2021.764512
Publication Year: 2021
Collection: Aarhus University: Research
Subject Terms: Genetics; Leu56Met; medullary thyroid cancer; multiple endocrine neoplasia type 2; RET
Description: Activating variants in the receptor tyrosine kinase REarranged during Transfection (RET) cause multiple endocrine neoplasia type 2 (MEN 2), an autosomal dominantly inherited cancer-susceptibility syndrome. The variant c.166C>A, p.Leu56Met in RET was recently reported in two patients with medullary thyroid cancer (MTC). The presence of a pheochromocytoma in one of the patients, suggested a possible pathogenic role of the variant in MEN 2A. Here, we present clinical follow up of a Danish RET Leu56Met cohort. Patients were evaluated for signs of MEN 2 according to a set of predefined criteria. None of the seven patients in our cohort exhibited evidence of MEN 2. Furthermore, we found the Leu56Met variant in our in-house diagnostic cohort with an allele frequency of 0.59%, suggesting that it is a common variant in the population. Additionally, none of the patients who harbored the allele were listed in the Danish MTC and MEN 2 registries. In conclusion, our findings do not support a pathogenic role of the Leu56Met variant in MEN 2.
Document Type: article in journal/newspaper
Language: English
ISSN: 1664-2392
Relation: info:eu-repo/semantics/altIdentifier/pmid/34925234; info:eu-repo/semantics/altIdentifier/pissn/1664-2392; info:eu-repo/semantics/altIdentifier/eissn/1664-2392
DOI: 10.3389/fendo.2021.764512
Availability: https://pure.au.dk/portal/en/publications/1c2a9ce9-a856-48b1-bdbf-a5151dbec3a3; https://doi.org/10.3389/fendo.2021.764512; https://www.scopus.com/pages/publications/85121397215
Rights: info:eu-repo/semantics/openAccess ; http://creativecommons.org/licenses/by/4.0/
Accession Number: edsbas.EF9EE569
Database: BASE