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Lamin A‐mediated nuclear lamina integrity is required for proper ciliogenesis

The primary cilium is a sensory organelle that receives specific signals from the extracellular environment important for vertebrate development and tissue homeostasis. Lamins, the major components of the nuclear lamina, are required to maintain the nuclear structure and are involved in most nuclear...

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Autores principales: Fan, Jia‐Rong, You, Li‐Ru, Wang, Won‐Jing, Huang, Wei‐Syun, Chu, Ching‐Tung, Chi, Ya‐Hui, Chen, Hong‐Chen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7534621/
https://www.ncbi.nlm.nih.gov/pubmed/32815283
http://dx.doi.org/10.15252/embr.201949680
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author Fan, Jia‐Rong
You, Li‐Ru
Wang, Won‐Jing
Huang, Wei‐Syun
Chu, Ching‐Tung
Chi, Ya‐Hui
Chen, Hong‐Chen
author_facet Fan, Jia‐Rong
You, Li‐Ru
Wang, Won‐Jing
Huang, Wei‐Syun
Chu, Ching‐Tung
Chi, Ya‐Hui
Chen, Hong‐Chen
author_sort Fan, Jia‐Rong
collection PubMed
description The primary cilium is a sensory organelle that receives specific signals from the extracellular environment important for vertebrate development and tissue homeostasis. Lamins, the major components of the nuclear lamina, are required to maintain the nuclear structure and are involved in most nuclear activities. In this study, we show that deficiency in lamin A/C causes defective ciliogenesis, accompanied by increased cytoplasmic accumulation of actin monomers and increased formation of actin filaments. Disruption of actin filaments by cytochalasin D rescues the defective ciliogenesis in lamin A/C‐depleted cells. Moreover, lamin A/C‐deficient cells display lower levels of nesprin 2 and defects in recruiting Arp2, myosin Va, and tau tubulin kinase 2 to the basal body during ciliogenesis. Collectively, our results uncover a functional link between nuclear lamina integrity and ciliogenesis and implicate the malfunction of primary cilia in the pathogenesis of laminopathy.
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spelling pubmed-75346212020-10-07 Lamin A‐mediated nuclear lamina integrity is required for proper ciliogenesis Fan, Jia‐Rong You, Li‐Ru Wang, Won‐Jing Huang, Wei‐Syun Chu, Ching‐Tung Chi, Ya‐Hui Chen, Hong‐Chen EMBO Rep Articles The primary cilium is a sensory organelle that receives specific signals from the extracellular environment important for vertebrate development and tissue homeostasis. Lamins, the major components of the nuclear lamina, are required to maintain the nuclear structure and are involved in most nuclear activities. In this study, we show that deficiency in lamin A/C causes defective ciliogenesis, accompanied by increased cytoplasmic accumulation of actin monomers and increased formation of actin filaments. Disruption of actin filaments by cytochalasin D rescues the defective ciliogenesis in lamin A/C‐depleted cells. Moreover, lamin A/C‐deficient cells display lower levels of nesprin 2 and defects in recruiting Arp2, myosin Va, and tau tubulin kinase 2 to the basal body during ciliogenesis. Collectively, our results uncover a functional link between nuclear lamina integrity and ciliogenesis and implicate the malfunction of primary cilia in the pathogenesis of laminopathy. John Wiley and Sons Inc. 2020-08-19 2020-10-05 /pmc/articles/PMC7534621/ /pubmed/32815283 http://dx.doi.org/10.15252/embr.201949680 Text en © 2020 The Authors. Published under the terms of the CC BY 4.0 license This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Articles
Fan, Jia‐Rong
You, Li‐Ru
Wang, Won‐Jing
Huang, Wei‐Syun
Chu, Ching‐Tung
Chi, Ya‐Hui
Chen, Hong‐Chen
Lamin A‐mediated nuclear lamina integrity is required for proper ciliogenesis
title Lamin A‐mediated nuclear lamina integrity is required for proper ciliogenesis
title_full Lamin A‐mediated nuclear lamina integrity is required for proper ciliogenesis
title_fullStr Lamin A‐mediated nuclear lamina integrity is required for proper ciliogenesis
title_full_unstemmed Lamin A‐mediated nuclear lamina integrity is required for proper ciliogenesis
title_short Lamin A‐mediated nuclear lamina integrity is required for proper ciliogenesis
title_sort lamin a‐mediated nuclear lamina integrity is required for proper ciliogenesis
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7534621/
https://www.ncbi.nlm.nih.gov/pubmed/32815283
http://dx.doi.org/10.15252/embr.201949680
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