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Developmental disruptions underlying brain abnormalities in ciliopathies
Primary cilia are essential conveyors of signals underlying major cell functions. Cerebral cortical progenitors and neurons have a primary cilium. The significance of cilia function for brain development and function is evident in the plethora of developmental brain disorders associated with human c...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Pub. Group
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4515781/ https://www.ncbi.nlm.nih.gov/pubmed/26206566 http://dx.doi.org/10.1038/ncomms8857 |
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author | Guo, Jiami Higginbotham, Holden Li, Jingjun Nichols, Jackie Hirt, Josua Ghukasyan, Vladimir Anton, E.S. |
author_facet | Guo, Jiami Higginbotham, Holden Li, Jingjun Nichols, Jackie Hirt, Josua Ghukasyan, Vladimir Anton, E.S. |
author_sort | Guo, Jiami |
collection | PubMed |
description | Primary cilia are essential conveyors of signals underlying major cell functions. Cerebral cortical progenitors and neurons have a primary cilium. The significance of cilia function for brain development and function is evident in the plethora of developmental brain disorders associated with human ciliopathies. Nevertheless, the role of primary cilia function in corticogenesis remains largely unknown. Here we delineate the functions of primary cilia in the construction of cerebral cortex and their relevance to ciliopathies, using an shRNA library targeting ciliopathy genes known to cause brain disorders, but whose roles in brain development are unclear. We used the library to query how ciliopathy genes affect distinct stages of mouse cortical development, in particular neural progenitor development, neuronal migration, neuronal differentiation and early neuronal connectivity. Our results define the developmental functions of ciliopathy genes and delineate disrupted developmental events that are integrally related to the emergence of brain abnormalities in ciliopathies. |
format | Online Article Text |
id | pubmed-4515781 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Nature Pub. Group |
record_format | MEDLINE/PubMed |
spelling | pubmed-45157812015-09-04 Developmental disruptions underlying brain abnormalities in ciliopathies Guo, Jiami Higginbotham, Holden Li, Jingjun Nichols, Jackie Hirt, Josua Ghukasyan, Vladimir Anton, E.S. Nat Commun Article Primary cilia are essential conveyors of signals underlying major cell functions. Cerebral cortical progenitors and neurons have a primary cilium. The significance of cilia function for brain development and function is evident in the plethora of developmental brain disorders associated with human ciliopathies. Nevertheless, the role of primary cilia function in corticogenesis remains largely unknown. Here we delineate the functions of primary cilia in the construction of cerebral cortex and their relevance to ciliopathies, using an shRNA library targeting ciliopathy genes known to cause brain disorders, but whose roles in brain development are unclear. We used the library to query how ciliopathy genes affect distinct stages of mouse cortical development, in particular neural progenitor development, neuronal migration, neuronal differentiation and early neuronal connectivity. Our results define the developmental functions of ciliopathy genes and delineate disrupted developmental events that are integrally related to the emergence of brain abnormalities in ciliopathies. Nature Pub. Group 2015-07-24 /pmc/articles/PMC4515781/ /pubmed/26206566 http://dx.doi.org/10.1038/ncomms8857 Text en Copyright © 2015, Nature Publishing Group, a division of Macmillan Publishers Limited. All Rights Reserved. http://creativecommons.org/licenses/by/4.0/ This work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third party material in this article are included in the article's Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/ |
spellingShingle | Article Guo, Jiami Higginbotham, Holden Li, Jingjun Nichols, Jackie Hirt, Josua Ghukasyan, Vladimir Anton, E.S. Developmental disruptions underlying brain abnormalities in ciliopathies |
title | Developmental disruptions underlying brain abnormalities in ciliopathies |
title_full | Developmental disruptions underlying brain abnormalities in ciliopathies |
title_fullStr | Developmental disruptions underlying brain abnormalities in ciliopathies |
title_full_unstemmed | Developmental disruptions underlying brain abnormalities in ciliopathies |
title_short | Developmental disruptions underlying brain abnormalities in ciliopathies |
title_sort | developmental disruptions underlying brain abnormalities in ciliopathies |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4515781/ https://www.ncbi.nlm.nih.gov/pubmed/26206566 http://dx.doi.org/10.1038/ncomms8857 |
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