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Novel age-dependent targets in vestibular schwannomas

BACKGROUND: Schwannomas are the most common neurofibromatosis type 2 (NF2)-associated tumors with significant phenotypic heterogeneity in patients. The most severe subtype has an early and rapid progression and the mild type has a later onset and a less aggressive course. The aim of this study was t...

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Autores principales: Toren, Amos, Reichardt, Juergen K, Andalibi, Ali, Hsu, Nancy Ya-Hsuan, Doherty, Joni, Slattery, William, Mehrian-Shai, Ruty
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4097955/
https://www.ncbi.nlm.nih.gov/pubmed/24980480
http://dx.doi.org/10.1186/1479-7364-8-10
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author Toren, Amos
Reichardt, Juergen K
Andalibi, Ali
Hsu, Nancy Ya-Hsuan
Doherty, Joni
Slattery, William
Mehrian-Shai, Ruty
author_facet Toren, Amos
Reichardt, Juergen K
Andalibi, Ali
Hsu, Nancy Ya-Hsuan
Doherty, Joni
Slattery, William
Mehrian-Shai, Ruty
author_sort Toren, Amos
collection PubMed
description BACKGROUND: Schwannomas are the most common neurofibromatosis type 2 (NF2)-associated tumors with significant phenotypic heterogeneity in patients. The most severe subtype has an early and rapid progression and the mild type has a later onset and a less aggressive course. The aim of this study was to elucidate the underlying molecular differences between these groups. We compared the gene expression pattern between patients with early to late age of onset. RESULTS: A gene signature of 21 genes was constructed to differentiate between early-onset and late-onset patients. We confirmed these results by real-time PCR for SNF1LK2, NGFRAP1L1 (BEX 5), GMNN, and EPHA2. CONCLUSION: Genes identified here may be additional aberrations in merlin-depleted cells that govern the disease onset. A significant number of these genes have been suggested as having a role in carcinogenesis and are used as biomarkers for prognosis in several other cancers. The role of these genes in NF2 carcinogenesis and their potential as biomarkers or drug target are worthwhile exploring.
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spelling pubmed-40979552014-07-16 Novel age-dependent targets in vestibular schwannomas Toren, Amos Reichardt, Juergen K Andalibi, Ali Hsu, Nancy Ya-Hsuan Doherty, Joni Slattery, William Mehrian-Shai, Ruty Hum Genomics Primary Research BACKGROUND: Schwannomas are the most common neurofibromatosis type 2 (NF2)-associated tumors with significant phenotypic heterogeneity in patients. The most severe subtype has an early and rapid progression and the mild type has a later onset and a less aggressive course. The aim of this study was to elucidate the underlying molecular differences between these groups. We compared the gene expression pattern between patients with early to late age of onset. RESULTS: A gene signature of 21 genes was constructed to differentiate between early-onset and late-onset patients. We confirmed these results by real-time PCR for SNF1LK2, NGFRAP1L1 (BEX 5), GMNN, and EPHA2. CONCLUSION: Genes identified here may be additional aberrations in merlin-depleted cells that govern the disease onset. A significant number of these genes have been suggested as having a role in carcinogenesis and are used as biomarkers for prognosis in several other cancers. The role of these genes in NF2 carcinogenesis and their potential as biomarkers or drug target are worthwhile exploring. BioMed Central 2014-06-30 /pmc/articles/PMC4097955/ /pubmed/24980480 http://dx.doi.org/10.1186/1479-7364-8-10 Text en Copyright © 2014 Toren et al.; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/4.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Primary Research
Toren, Amos
Reichardt, Juergen K
Andalibi, Ali
Hsu, Nancy Ya-Hsuan
Doherty, Joni
Slattery, William
Mehrian-Shai, Ruty
Novel age-dependent targets in vestibular schwannomas
title Novel age-dependent targets in vestibular schwannomas
title_full Novel age-dependent targets in vestibular schwannomas
title_fullStr Novel age-dependent targets in vestibular schwannomas
title_full_unstemmed Novel age-dependent targets in vestibular schwannomas
title_short Novel age-dependent targets in vestibular schwannomas
title_sort novel age-dependent targets in vestibular schwannomas
topic Primary Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4097955/
https://www.ncbi.nlm.nih.gov/pubmed/24980480
http://dx.doi.org/10.1186/1479-7364-8-10
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