Epigenetic Mechanisms in Hirschsprung Disease
Hirschsprung disease (HSCR, OMIM 142623) is due to a failure of enteric precursor cells derived from neural crest (EPCs) to proliferate, migrate, survive or differentiate during Enteric Nervous System (ENS) formation. This is a complex process which requires a strict regulation that results in an EN...
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doaj-8e92074460df4e17a9b76ea303e8fa022020-11-24T22:09:22ZengMDPI AGInternational Journal of Molecular Sciences1422-00672019-06-012013312310.3390/ijms20133123ijms20133123Epigenetic Mechanisms in Hirschsprung DiseaseAna Torroglosa0Leticia Villalba-Benito1Berta Luzón-Toro2Raquel María Fernández3Guillermo Antiñolo4Salud Borrego5Department of Maternofetal Medicine, Genetics and Reproduction, Institute of Biomedicine of Seville (IBIS), University Hospital Virgen del Rocío/CSIC/University of Seville, 41013 Seville, SpainDepartment of Maternofetal Medicine, Genetics and Reproduction, Institute of Biomedicine of Seville (IBIS), University Hospital Virgen del Rocío/CSIC/University of Seville, 41013 Seville, SpainDepartment of Maternofetal Medicine, Genetics and Reproduction, Institute of Biomedicine of Seville (IBIS), University Hospital Virgen del Rocío/CSIC/University of Seville, 41013 Seville, SpainDepartment of Maternofetal Medicine, Genetics and Reproduction, Institute of Biomedicine of Seville (IBIS), University Hospital Virgen del Rocío/CSIC/University of Seville, 41013 Seville, SpainDepartment of Maternofetal Medicine, Genetics and Reproduction, Institute of Biomedicine of Seville (IBIS), University Hospital Virgen del Rocío/CSIC/University of Seville, 41013 Seville, SpainDepartment of Maternofetal Medicine, Genetics and Reproduction, Institute of Biomedicine of Seville (IBIS), University Hospital Virgen del Rocío/CSIC/University of Seville, 41013 Seville, SpainHirschsprung disease (HSCR, OMIM 142623) is due to a failure of enteric precursor cells derived from neural crest (EPCs) to proliferate, migrate, survive or differentiate during Enteric Nervous System (ENS) formation. This is a complex process which requires a strict regulation that results in an ENS specific gene expression pattern. Alterations at this level lead to the onset of neurocristopathies such as HSCR. Gene expression is regulated by different mechanisms, such as DNA modifications (at the epigenetic level), transcriptional mechanisms (transcription factors, silencers, enhancers and repressors), postranscriptional mechanisms (3′UTR and ncRNA) and regulation of translation. All these mechanisms are finally implicated in cell signaling to determine the migration, proliferation, differentiation and survival processes for correct ENS development. In this review, we have performed an overview on the role of epigenetic mechanisms at transcriptional and posttranscriptional levels on these cellular events in neural crest cells (NCCs), ENS development, as well as in HSCR.https://www.mdpi.com/1422-0067/20/13/3123enteric nervous system developmentHirschsprung diseaseneural crest cellsepigenetic mechanisms |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Ana Torroglosa Leticia Villalba-Benito Berta Luzón-Toro Raquel María Fernández Guillermo Antiñolo Salud Borrego |
spellingShingle |
Ana Torroglosa Leticia Villalba-Benito Berta Luzón-Toro Raquel María Fernández Guillermo Antiñolo Salud Borrego Epigenetic Mechanisms in Hirschsprung Disease International Journal of Molecular Sciences enteric nervous system development Hirschsprung disease neural crest cells epigenetic mechanisms |
author_facet |
Ana Torroglosa Leticia Villalba-Benito Berta Luzón-Toro Raquel María Fernández Guillermo Antiñolo Salud Borrego |
author_sort |
Ana Torroglosa |
title |
Epigenetic Mechanisms in Hirschsprung Disease |
title_short |
Epigenetic Mechanisms in Hirschsprung Disease |
title_full |
Epigenetic Mechanisms in Hirschsprung Disease |
title_fullStr |
Epigenetic Mechanisms in Hirschsprung Disease |
title_full_unstemmed |
Epigenetic Mechanisms in Hirschsprung Disease |
title_sort |
epigenetic mechanisms in hirschsprung disease |
publisher |
MDPI AG |
series |
International Journal of Molecular Sciences |
issn |
1422-0067 |
publishDate |
2019-06-01 |
description |
Hirschsprung disease (HSCR, OMIM 142623) is due to a failure of enteric precursor cells derived from neural crest (EPCs) to proliferate, migrate, survive or differentiate during Enteric Nervous System (ENS) formation. This is a complex process which requires a strict regulation that results in an ENS specific gene expression pattern. Alterations at this level lead to the onset of neurocristopathies such as HSCR. Gene expression is regulated by different mechanisms, such as DNA modifications (at the epigenetic level), transcriptional mechanisms (transcription factors, silencers, enhancers and repressors), postranscriptional mechanisms (3′UTR and ncRNA) and regulation of translation. All these mechanisms are finally implicated in cell signaling to determine the migration, proliferation, differentiation and survival processes for correct ENS development. In this review, we have performed an overview on the role of epigenetic mechanisms at transcriptional and posttranscriptional levels on these cellular events in neural crest cells (NCCs), ENS development, as well as in HSCR. |
topic |
enteric nervous system development Hirschsprung disease neural crest cells epigenetic mechanisms |
url |
https://www.mdpi.com/1422-0067/20/13/3123 |
work_keys_str_mv |
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1725812240721903616 |