A Focus on Regulatory Networks Linking MicroRNAs, Transcription Factors and Target Genes in Neuroblastoma

Neuroblastoma (NB) is a tumor of the peripheral sympathetic nervous system that substantially contributes to childhood cancer mortality. NB originates from neural crest cells (NCCs) undergoing a defective sympathetic neuronal differentiation and although the starting events leading to the developmen...

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Autores principales:	Patrizia Perri, Mirco Ponzoni, Maria Valeria Corrias, Isabella Ceccherini, Simona Candiani, Tiziana Bachetti
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	neuroblastoma microRNAs transcription factors miR-34 let-7 miR-204 Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
Acceso en línea:	https://doaj.org/article/54c5db33768c46fca095c44075844115
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spelling	oai:doaj.org-article:54c5db33768c46fca095c440758441152021-11-11T15:33:55ZA Focus on Regulatory Networks Linking MicroRNAs, Transcription Factors and Target Genes in Neuroblastoma10.3390/cancers132155282072-6694https://doaj.org/article/54c5db33768c46fca095c440758441152021-11-01T00:00:00Zhttps://www.mdpi.com/2072-6694/13/21/5528https://doaj.org/toc/2072-6694Neuroblastoma (NB) is a tumor of the peripheral sympathetic nervous system that substantially contributes to childhood cancer mortality. NB originates from neural crest cells (NCCs) undergoing a defective sympathetic neuronal differentiation and although the starting events leading to the development of NB remain to be fully elucidated, the master role of genetic alterations in key oncogenes has been ascertained: (1) amplification and/or over-expression of <i>MYCN</i>, which is strongly associated with tumor progression and invasion; (2) activating mutations, amplification and/or over-expression of <i>ALK</i>, which is involved in tumor initiation, angiogenesis and invasion; (3) amplification and/or over-expression of <i>LIN28B</i>, promoting proliferation and suppression of neuroblast differentiation; (4) mutations and/or over-expression of <i>PHOX2B</i>, which is involved in the regulation of NB differentiation, stemness maintenance, migration and metastasis. Moreover, altered microRNA (miRNA) expression takes part in generating pathogenetic networks, in which the regulatory loops among transcription factors, miRNAs and target genes lead to complex and aberrant oncogene expression that underlies the development of a tumor. In this review, we have focused on the circuitry linking the oncogenic transcription factors MYCN and PHOX2B with their transcriptional targets ALK and LIN28B and the tumor suppressor microRNAs let-7, miR-34 and miR-204, which should act as down-regulators of their expression. We have also looked at the physiologic role of these genetic and epigenetic determinants in NC development, as well as in terminal differentiation, with their pathogenic dysregulation leading to NB oncogenesis.Patrizia PerriMirco PonzoniMaria Valeria CorriasIsabella CeccheriniSimona CandianiTiziana BachettiMDPI AGarticleneuroblastomamicroRNAstranscription factorsmiR-34let-7miR-204Neoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENCancers, Vol 13, Iss 5528, p 5528 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	neuroblastoma microRNAs transcription factors miR-34 let-7 miR-204 Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
spellingShingle	neuroblastoma microRNAs transcription factors miR-34 let-7 miR-204 Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282 Patrizia Perri Mirco Ponzoni Maria Valeria Corrias Isabella Ceccherini Simona Candiani Tiziana Bachetti A Focus on Regulatory Networks Linking MicroRNAs, Transcription Factors and Target Genes in Neuroblastoma
description	Neuroblastoma (NB) is a tumor of the peripheral sympathetic nervous system that substantially contributes to childhood cancer mortality. NB originates from neural crest cells (NCCs) undergoing a defective sympathetic neuronal differentiation and although the starting events leading to the development of NB remain to be fully elucidated, the master role of genetic alterations in key oncogenes has been ascertained: (1) amplification and/or over-expression of <i>MYCN</i>, which is strongly associated with tumor progression and invasion; (2) activating mutations, amplification and/or over-expression of <i>ALK</i>, which is involved in tumor initiation, angiogenesis and invasion; (3) amplification and/or over-expression of <i>LIN28B</i>, promoting proliferation and suppression of neuroblast differentiation; (4) mutations and/or over-expression of <i>PHOX2B</i>, which is involved in the regulation of NB differentiation, stemness maintenance, migration and metastasis. Moreover, altered microRNA (miRNA) expression takes part in generating pathogenetic networks, in which the regulatory loops among transcription factors, miRNAs and target genes lead to complex and aberrant oncogene expression that underlies the development of a tumor. In this review, we have focused on the circuitry linking the oncogenic transcription factors MYCN and PHOX2B with their transcriptional targets ALK and LIN28B and the tumor suppressor microRNAs let-7, miR-34 and miR-204, which should act as down-regulators of their expression. We have also looked at the physiologic role of these genetic and epigenetic determinants in NC development, as well as in terminal differentiation, with their pathogenic dysregulation leading to NB oncogenesis.
format	article
author	Patrizia Perri Mirco Ponzoni Maria Valeria Corrias Isabella Ceccherini Simona Candiani Tiziana Bachetti
author_facet	Patrizia Perri Mirco Ponzoni Maria Valeria Corrias Isabella Ceccherini Simona Candiani Tiziana Bachetti
author_sort	Patrizia Perri
title	A Focus on Regulatory Networks Linking MicroRNAs, Transcription Factors and Target Genes in Neuroblastoma
title_short	A Focus on Regulatory Networks Linking MicroRNAs, Transcription Factors and Target Genes in Neuroblastoma
title_full	A Focus on Regulatory Networks Linking MicroRNAs, Transcription Factors and Target Genes in Neuroblastoma
title_fullStr	A Focus on Regulatory Networks Linking MicroRNAs, Transcription Factors and Target Genes in Neuroblastoma
title_full_unstemmed	A Focus on Regulatory Networks Linking MicroRNAs, Transcription Factors and Target Genes in Neuroblastoma
title_sort	focus on regulatory networks linking micrornas, transcription factors and target genes in neuroblastoma
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/54c5db33768c46fca095c44075844115
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A Focus on Regulatory Networks Linking MicroRNAs, Transcription Factors and Target Genes in Neuroblastoma

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