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Long Noncoding RNAs: Emerging Players in Medulloblastoma

Central Nervous System tumors are the leading cause of cancer-related death in children, and medulloblastoma has the highest incidence rate. The current therapies achieve a 5-year survival rate of 50–80%, but often inflict severe secondary effects demanding the urgent development of novel, effective...

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Autores principales: Laneve, Pietro, Rea, Jessica, Caffarelli, Elisa
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6426782/
https://www.ncbi.nlm.nih.gov/pubmed/30923703
http://dx.doi.org/10.3389/fped.2019.00067
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author Laneve, Pietro
Rea, Jessica
Caffarelli, Elisa
author_facet Laneve, Pietro
Rea, Jessica
Caffarelli, Elisa
author_sort Laneve, Pietro
collection PubMed
description Central Nervous System tumors are the leading cause of cancer-related death in children, and medulloblastoma has the highest incidence rate. The current therapies achieve a 5-year survival rate of 50–80%, but often inflict severe secondary effects demanding the urgent development of novel, effective, and less toxic therapeutic strategies. Historically identified on a histopathological basis, medulloblastoma was later classified into four major subgroups—namely WNT, SHH, Group 3, and Group 4—each characterized by distinct transcriptional profiles, copy-number aberrations, somatic mutations, and clinical outcomes. Additional complexity was recently provided by integrating gene- and non-gene-based data, which indicates that each subclass can be further subdivided into specific subtypes. These deeper classifications, while getting over the typical tumor heterogeneity, indicate that different forms of medulloblastoma hold different molecular drivers that can be successfully exploited for a greater diagnostic accuracy and for the development of novel, targeted treatments. Long noncoding RNAs are transcripts that lack coding potential and play relevant roles as regulators of gene expression in mammalian differentiation and developmental processes. Their cell type- and tissue-specificity, higher than mRNAs, make them more informative about cell- type identity than protein-coding genes. Remarkably, about 40% of long noncoding RNAs are expressed in the brain and their aberrant expression has been linked to neuro-oncological disorders. However, while their involvement in gliomas and neuroblastomas has been extensively studied, their role in medulloblastoma is still poorly explored. Here, we present an overview of current knowledge regarding the function played by long noncoding RNAs in medulloblastoma biology.
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spelling pubmed-64267822019-03-28 Long Noncoding RNAs: Emerging Players in Medulloblastoma Laneve, Pietro Rea, Jessica Caffarelli, Elisa Front Pediatr Pediatrics Central Nervous System tumors are the leading cause of cancer-related death in children, and medulloblastoma has the highest incidence rate. The current therapies achieve a 5-year survival rate of 50–80%, but often inflict severe secondary effects demanding the urgent development of novel, effective, and less toxic therapeutic strategies. Historically identified on a histopathological basis, medulloblastoma was later classified into four major subgroups—namely WNT, SHH, Group 3, and Group 4—each characterized by distinct transcriptional profiles, copy-number aberrations, somatic mutations, and clinical outcomes. Additional complexity was recently provided by integrating gene- and non-gene-based data, which indicates that each subclass can be further subdivided into specific subtypes. These deeper classifications, while getting over the typical tumor heterogeneity, indicate that different forms of medulloblastoma hold different molecular drivers that can be successfully exploited for a greater diagnostic accuracy and for the development of novel, targeted treatments. Long noncoding RNAs are transcripts that lack coding potential and play relevant roles as regulators of gene expression in mammalian differentiation and developmental processes. Their cell type- and tissue-specificity, higher than mRNAs, make them more informative about cell- type identity than protein-coding genes. Remarkably, about 40% of long noncoding RNAs are expressed in the brain and their aberrant expression has been linked to neuro-oncological disorders. However, while their involvement in gliomas and neuroblastomas has been extensively studied, their role in medulloblastoma is still poorly explored. Here, we present an overview of current knowledge regarding the function played by long noncoding RNAs in medulloblastoma biology. Frontiers Media S.A. 2019-03-14 /pmc/articles/PMC6426782/ /pubmed/30923703 http://dx.doi.org/10.3389/fped.2019.00067 Text en Copyright © 2019 Laneve, Rea and Caffarelli. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Pediatrics
Laneve, Pietro
Rea, Jessica
Caffarelli, Elisa
Long Noncoding RNAs: Emerging Players in Medulloblastoma
title Long Noncoding RNAs: Emerging Players in Medulloblastoma
title_full Long Noncoding RNAs: Emerging Players in Medulloblastoma
title_fullStr Long Noncoding RNAs: Emerging Players in Medulloblastoma
title_full_unstemmed Long Noncoding RNAs: Emerging Players in Medulloblastoma
title_short Long Noncoding RNAs: Emerging Players in Medulloblastoma
title_sort long noncoding rnas: emerging players in medulloblastoma
topic Pediatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6426782/
https://www.ncbi.nlm.nih.gov/pubmed/30923703
http://dx.doi.org/10.3389/fped.2019.00067
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