Cargando…
Updates on the Role of Molecular Alterations and NOTCH Signalling in the Development of Neuroendocrine Neoplasms
Neuroendocrine neoplasms (NENs) comprise a heterogeneous group of rare malignancies, mainly originating from hormone-secreting cells, which are widespread in human tissues. The identification of mutations in ATRX/DAXX genes in sporadic NENs, as well as the high burden of mutations scattered througho...
Autores principales: | , , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2019
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6780206/ https://www.ncbi.nlm.nih.gov/pubmed/31443481 http://dx.doi.org/10.3390/jcm8091277 |
_version_ | 1783457076153942016 |
---|---|
author | von Arx, Claudia Capozzi, Monica López-Jiménez, Elena Ottaiano, Alessandro Tatangelo, Fabiana Di Mauro, Annabella Nasti, Guglielmo Tornesello, Maria Lina Tafuto, Salvatore |
author_facet | von Arx, Claudia Capozzi, Monica López-Jiménez, Elena Ottaiano, Alessandro Tatangelo, Fabiana Di Mauro, Annabella Nasti, Guglielmo Tornesello, Maria Lina Tafuto, Salvatore |
author_sort | von Arx, Claudia |
collection | PubMed |
description | Neuroendocrine neoplasms (NENs) comprise a heterogeneous group of rare malignancies, mainly originating from hormone-secreting cells, which are widespread in human tissues. The identification of mutations in ATRX/DAXX genes in sporadic NENs, as well as the high burden of mutations scattered throughout the multiple endocrine neoplasia type 1 (MEN-1) gene in both sporadic and inherited syndromes, provided new insights into the molecular biology of tumour development. Other molecular mechanisms, such as the NOTCH signalling pathway, have shown to play an important role in the pathogenesis of NENs. NOTCH receptors are expressed on neuroendocrine cells and generally act as tumour suppressor proteins, but in some contexts can function as oncogenes. The biological heterogeneity of NENs suggests that to fully understand the role and the potential therapeutic implications of gene mutations and NOTCH signalling in NENs, a comprehensive analysis of genetic alterations, NOTCH expression patterns and their potential role across all NEN subtypes is required. |
format | Online Article Text |
id | pubmed-6780206 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-67802062019-10-30 Updates on the Role of Molecular Alterations and NOTCH Signalling in the Development of Neuroendocrine Neoplasms von Arx, Claudia Capozzi, Monica López-Jiménez, Elena Ottaiano, Alessandro Tatangelo, Fabiana Di Mauro, Annabella Nasti, Guglielmo Tornesello, Maria Lina Tafuto, Salvatore J Clin Med Review Neuroendocrine neoplasms (NENs) comprise a heterogeneous group of rare malignancies, mainly originating from hormone-secreting cells, which are widespread in human tissues. The identification of mutations in ATRX/DAXX genes in sporadic NENs, as well as the high burden of mutations scattered throughout the multiple endocrine neoplasia type 1 (MEN-1) gene in both sporadic and inherited syndromes, provided new insights into the molecular biology of tumour development. Other molecular mechanisms, such as the NOTCH signalling pathway, have shown to play an important role in the pathogenesis of NENs. NOTCH receptors are expressed on neuroendocrine cells and generally act as tumour suppressor proteins, but in some contexts can function as oncogenes. The biological heterogeneity of NENs suggests that to fully understand the role and the potential therapeutic implications of gene mutations and NOTCH signalling in NENs, a comprehensive analysis of genetic alterations, NOTCH expression patterns and their potential role across all NEN subtypes is required. MDPI 2019-08-22 /pmc/articles/PMC6780206/ /pubmed/31443481 http://dx.doi.org/10.3390/jcm8091277 Text en © 2019 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review von Arx, Claudia Capozzi, Monica López-Jiménez, Elena Ottaiano, Alessandro Tatangelo, Fabiana Di Mauro, Annabella Nasti, Guglielmo Tornesello, Maria Lina Tafuto, Salvatore Updates on the Role of Molecular Alterations and NOTCH Signalling in the Development of Neuroendocrine Neoplasms |
title | Updates on the Role of Molecular Alterations and NOTCH Signalling in the Development of Neuroendocrine Neoplasms |
title_full | Updates on the Role of Molecular Alterations and NOTCH Signalling in the Development of Neuroendocrine Neoplasms |
title_fullStr | Updates on the Role of Molecular Alterations and NOTCH Signalling in the Development of Neuroendocrine Neoplasms |
title_full_unstemmed | Updates on the Role of Molecular Alterations and NOTCH Signalling in the Development of Neuroendocrine Neoplasms |
title_short | Updates on the Role of Molecular Alterations and NOTCH Signalling in the Development of Neuroendocrine Neoplasms |
title_sort | updates on the role of molecular alterations and notch signalling in the development of neuroendocrine neoplasms |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6780206/ https://www.ncbi.nlm.nih.gov/pubmed/31443481 http://dx.doi.org/10.3390/jcm8091277 |
work_keys_str_mv | AT vonarxclaudia updatesontheroleofmolecularalterationsandnotchsignallinginthedevelopmentofneuroendocrineneoplasms AT capozzimonica updatesontheroleofmolecularalterationsandnotchsignallinginthedevelopmentofneuroendocrineneoplasms AT lopezjimenezelena updatesontheroleofmolecularalterationsandnotchsignallinginthedevelopmentofneuroendocrineneoplasms AT ottaianoalessandro updatesontheroleofmolecularalterationsandnotchsignallinginthedevelopmentofneuroendocrineneoplasms AT tatangelofabiana updatesontheroleofmolecularalterationsandnotchsignallinginthedevelopmentofneuroendocrineneoplasms AT dimauroannabella updatesontheroleofmolecularalterationsandnotchsignallinginthedevelopmentofneuroendocrineneoplasms AT nastiguglielmo updatesontheroleofmolecularalterationsandnotchsignallinginthedevelopmentofneuroendocrineneoplasms AT tornesellomarialina updatesontheroleofmolecularalterationsandnotchsignallinginthedevelopmentofneuroendocrineneoplasms AT tafutosalvatore updatesontheroleofmolecularalterationsandnotchsignallinginthedevelopmentofneuroendocrineneoplasms |