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Primary Ciliary Deficits in the Dentate Gyrus of Fragile X Syndrome
The primary cilium is the non-motile cilium present in most mammalian cell types and functions as an antenna for cells to sense signals. Ablating primary cilia in postnatal newborn neurons of the dentate gyrus (DG) results in both reduced dendritic arborization and synaptic strength, leading to hipp...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7419715/ https://www.ncbi.nlm.nih.gov/pubmed/32735823 http://dx.doi.org/10.1016/j.stemcr.2020.07.001 |
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author | Lee, Bumwhee Panda, Shree Lee, Hye Young |
author_facet | Lee, Bumwhee Panda, Shree Lee, Hye Young |
author_sort | Lee, Bumwhee |
collection | PubMed |
description | The primary cilium is the non-motile cilium present in most mammalian cell types and functions as an antenna for cells to sense signals. Ablating primary cilia in postnatal newborn neurons of the dentate gyrus (DG) results in both reduced dendritic arborization and synaptic strength, leading to hippocampal-dependent learning and memory deficits. Fragile X syndrome (FXS) is a common form of inheritance for intellectual disabilities with a high risk for autism spectrum disorders, and Fmr1 KO mice, a mouse model for FXS, demonstrate deficits in newborn neuron differentiation, dendritic morphology, and memory formation in the DG. Here, we found that the number of primary cilia in Fmr1 KO mice is reduced, specifically in the DG of the hippocampus. Moreover, this cilia loss was observed postnatally mainly in newborn neurons generated from the DG, implicating that these primary ciliary deficits may possibly contribute to the pathophysiology of FXS. |
format | Online Article Text |
id | pubmed-7419715 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-74197152020-08-14 Primary Ciliary Deficits in the Dentate Gyrus of Fragile X Syndrome Lee, Bumwhee Panda, Shree Lee, Hye Young Stem Cell Reports Article The primary cilium is the non-motile cilium present in most mammalian cell types and functions as an antenna for cells to sense signals. Ablating primary cilia in postnatal newborn neurons of the dentate gyrus (DG) results in both reduced dendritic arborization and synaptic strength, leading to hippocampal-dependent learning and memory deficits. Fragile X syndrome (FXS) is a common form of inheritance for intellectual disabilities with a high risk for autism spectrum disorders, and Fmr1 KO mice, a mouse model for FXS, demonstrate deficits in newborn neuron differentiation, dendritic morphology, and memory formation in the DG. Here, we found that the number of primary cilia in Fmr1 KO mice is reduced, specifically in the DG of the hippocampus. Moreover, this cilia loss was observed postnatally mainly in newborn neurons generated from the DG, implicating that these primary ciliary deficits may possibly contribute to the pathophysiology of FXS. Elsevier 2020-07-30 /pmc/articles/PMC7419715/ /pubmed/32735823 http://dx.doi.org/10.1016/j.stemcr.2020.07.001 Text en © 2020 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Article Lee, Bumwhee Panda, Shree Lee, Hye Young Primary Ciliary Deficits in the Dentate Gyrus of Fragile X Syndrome |
title | Primary Ciliary Deficits in the Dentate Gyrus of Fragile X Syndrome |
title_full | Primary Ciliary Deficits in the Dentate Gyrus of Fragile X Syndrome |
title_fullStr | Primary Ciliary Deficits in the Dentate Gyrus of Fragile X Syndrome |
title_full_unstemmed | Primary Ciliary Deficits in the Dentate Gyrus of Fragile X Syndrome |
title_short | Primary Ciliary Deficits in the Dentate Gyrus of Fragile X Syndrome |
title_sort | primary ciliary deficits in the dentate gyrus of fragile x syndrome |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7419715/ https://www.ncbi.nlm.nih.gov/pubmed/32735823 http://dx.doi.org/10.1016/j.stemcr.2020.07.001 |
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