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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...

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Detalles Bibliográficos
Autores principales: Lee, Bumwhee, Panda, Shree, Lee, Hye Young
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
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.
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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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