author_facet Li, Sanming
Zhou, Jing
Zhang, Liying
Li, Juan
Yu, Jingwen
Ning, Ke
Qu, Yangluowa
He, Hui
Chen, Yongxiong
Reinach, Peter S.
Liu, Chia‐Yang
Liu, Zuguo
Li, Wei
Li, Sanming
Zhou, Jing
Zhang, Liying
Li, Juan
Yu, Jingwen
Ning, Ke
Qu, Yangluowa
He, Hui
Chen, Yongxiong
Reinach, Peter S.
Liu, Chia‐Yang
Liu, Zuguo
Li, Wei
author Li, Sanming
Zhou, Jing
Zhang, Liying
Li, Juan
Yu, Jingwen
Ning, Ke
Qu, Yangluowa
He, Hui
Chen, Yongxiong
Reinach, Peter S.
Liu, Chia‐Yang
Liu, Zuguo
Li, Wei
spellingShingle Li, Sanming
Zhou, Jing
Zhang, Liying
Li, Juan
Yu, Jingwen
Ning, Ke
Qu, Yangluowa
He, Hui
Chen, Yongxiong
Reinach, Peter S.
Liu, Chia‐Yang
Liu, Zuguo
Li, Wei
Journal of Cellular and Molecular Medicine
Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway
Cell Biology
Molecular Medicine
author_sort li, sanming
spelling Li, Sanming Zhou, Jing Zhang, Liying Li, Juan Yu, Jingwen Ning, Ke Qu, Yangluowa He, Hui Chen, Yongxiong Reinach, Peter S. Liu, Chia‐Yang Liu, Zuguo Li, Wei 1582-1838 1582-4934 Wiley Cell Biology Molecular Medicine http://dx.doi.org/10.1111/jcmm.13311 <jats:title>Abstract</jats:title><jats:p>Ectodysplasin A (Eda), a member of the tumour necrosis factor superfamily, plays an important role in ectodermal organ development. An <jats:italic><jats:styled-content style="fixed-case">EDA</jats:styled-content></jats:italic> mutation underlies the most common of ectodermal dysplasias, that is X‐linked hypohidrotic ectodermal dysplasia (<jats:styled-content style="fixed-case">XLHED</jats:styled-content>) in humans. Even though it lacks a developmental function, the role of Eda during the postnatal stage remains elusive. In this study, we found tight junctional proteins <jats:styled-content style="fixed-case">ZO</jats:styled-content>‐1 and claudin‐1 expression is largely reduced in epidermal, corneal and lung epithelia in <jats:italic>Eda</jats:italic> mutant <jats:italic>Tabby</jats:italic> mice at different postnatal ages. These declines are associated with tail ulceration, corneal pannus formation and lung infection. Furthermore, topical application of recombinant Eda protein markedly mitigated corneal barrier dysfunction. Using cultures of a human corneal epithelial cell line and <jats:italic>Tabby</jats:italic> mouse skin tissue explants, Eda up‐regulated expression of <jats:styled-content style="fixed-case">ZO</jats:styled-content>‐1 and claudin‐1 through activation of the sonic hedgehog signalling pathway. We conclude that <jats:italic><jats:styled-content style="fixed-case">EDA</jats:styled-content></jats:italic> gene expression contributes to the maintenance of epithelial barrier function. Such insight may help efforts to identify novel strategies for improving management of <jats:styled-content style="fixed-case">XLHED</jats:styled-content> disease manifestations in a clinical setting.</jats:p> Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway Journal of Cellular and Molecular Medicine
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title Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway
title_unstemmed Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway
title_full Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway
title_fullStr Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway
title_full_unstemmed Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway
title_short Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway
title_sort ectodysplasin a regulates epithelial barrier function through sonic hedgehog signalling pathway
topic Cell Biology
Molecular Medicine
url http://dx.doi.org/10.1111/jcmm.13311
publishDate 2018
physical 230-240
description <jats:title>Abstract</jats:title><jats:p>Ectodysplasin A (Eda), a member of the tumour necrosis factor superfamily, plays an important role in ectodermal organ development. An <jats:italic><jats:styled-content style="fixed-case">EDA</jats:styled-content></jats:italic> mutation underlies the most common of ectodermal dysplasias, that is X‐linked hypohidrotic ectodermal dysplasia (<jats:styled-content style="fixed-case">XLHED</jats:styled-content>) in humans. Even though it lacks a developmental function, the role of Eda during the postnatal stage remains elusive. In this study, we found tight junctional proteins <jats:styled-content style="fixed-case">ZO</jats:styled-content>‐1 and claudin‐1 expression is largely reduced in epidermal, corneal and lung epithelia in <jats:italic>Eda</jats:italic> mutant <jats:italic>Tabby</jats:italic> mice at different postnatal ages. These declines are associated with tail ulceration, corneal pannus formation and lung infection. Furthermore, topical application of recombinant Eda protein markedly mitigated corneal barrier dysfunction. Using cultures of a human corneal epithelial cell line and <jats:italic>Tabby</jats:italic> mouse skin tissue explants, Eda up‐regulated expression of <jats:styled-content style="fixed-case">ZO</jats:styled-content>‐1 and claudin‐1 through activation of the sonic hedgehog signalling pathway. We conclude that <jats:italic><jats:styled-content style="fixed-case">EDA</jats:styled-content></jats:italic> gene expression contributes to the maintenance of epithelial barrier function. Such insight may help efforts to identify novel strategies for improving management of <jats:styled-content style="fixed-case">XLHED</jats:styled-content> disease manifestations in a clinical setting.</jats:p>
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author Li, Sanming, Zhou, Jing, Zhang, Liying, Li, Juan, Yu, Jingwen, Ning, Ke, Qu, Yangluowa, He, Hui, Chen, Yongxiong, Reinach, Peter S., Liu, Chia‐Yang, Liu, Zuguo, Li, Wei
author_facet Li, Sanming, Zhou, Jing, Zhang, Liying, Li, Juan, Yu, Jingwen, Ning, Ke, Qu, Yangluowa, He, Hui, Chen, Yongxiong, Reinach, Peter S., Liu, Chia‐Yang, Liu, Zuguo, Li, Wei, Li, Sanming, Zhou, Jing, Zhang, Liying, Li, Juan, Yu, Jingwen, Ning, Ke, Qu, Yangluowa, He, Hui, Chen, Yongxiong, Reinach, Peter S., Liu, Chia‐Yang, Liu, Zuguo, Li, Wei
author_sort li, sanming
container_issue 1
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container_title Journal of Cellular and Molecular Medicine
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description <jats:title>Abstract</jats:title><jats:p>Ectodysplasin A (Eda), a member of the tumour necrosis factor superfamily, plays an important role in ectodermal organ development. An <jats:italic><jats:styled-content style="fixed-case">EDA</jats:styled-content></jats:italic> mutation underlies the most common of ectodermal dysplasias, that is X‐linked hypohidrotic ectodermal dysplasia (<jats:styled-content style="fixed-case">XLHED</jats:styled-content>) in humans. Even though it lacks a developmental function, the role of Eda during the postnatal stage remains elusive. In this study, we found tight junctional proteins <jats:styled-content style="fixed-case">ZO</jats:styled-content>‐1 and claudin‐1 expression is largely reduced in epidermal, corneal and lung epithelia in <jats:italic>Eda</jats:italic> mutant <jats:italic>Tabby</jats:italic> mice at different postnatal ages. These declines are associated with tail ulceration, corneal pannus formation and lung infection. Furthermore, topical application of recombinant Eda protein markedly mitigated corneal barrier dysfunction. Using cultures of a human corneal epithelial cell line and <jats:italic>Tabby</jats:italic> mouse skin tissue explants, Eda up‐regulated expression of <jats:styled-content style="fixed-case">ZO</jats:styled-content>‐1 and claudin‐1 through activation of the sonic hedgehog signalling pathway. We conclude that <jats:italic><jats:styled-content style="fixed-case">EDA</jats:styled-content></jats:italic> gene expression contributes to the maintenance of epithelial barrier function. Such insight may help efforts to identify novel strategies for improving management of <jats:styled-content style="fixed-case">XLHED</jats:styled-content> disease manifestations in a clinical setting.</jats:p>
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spelling Li, Sanming Zhou, Jing Zhang, Liying Li, Juan Yu, Jingwen Ning, Ke Qu, Yangluowa He, Hui Chen, Yongxiong Reinach, Peter S. Liu, Chia‐Yang Liu, Zuguo Li, Wei 1582-1838 1582-4934 Wiley Cell Biology Molecular Medicine http://dx.doi.org/10.1111/jcmm.13311 <jats:title>Abstract</jats:title><jats:p>Ectodysplasin A (Eda), a member of the tumour necrosis factor superfamily, plays an important role in ectodermal organ development. An <jats:italic><jats:styled-content style="fixed-case">EDA</jats:styled-content></jats:italic> mutation underlies the most common of ectodermal dysplasias, that is X‐linked hypohidrotic ectodermal dysplasia (<jats:styled-content style="fixed-case">XLHED</jats:styled-content>) in humans. Even though it lacks a developmental function, the role of Eda during the postnatal stage remains elusive. In this study, we found tight junctional proteins <jats:styled-content style="fixed-case">ZO</jats:styled-content>‐1 and claudin‐1 expression is largely reduced in epidermal, corneal and lung epithelia in <jats:italic>Eda</jats:italic> mutant <jats:italic>Tabby</jats:italic> mice at different postnatal ages. These declines are associated with tail ulceration, corneal pannus formation and lung infection. Furthermore, topical application of recombinant Eda protein markedly mitigated corneal barrier dysfunction. Using cultures of a human corneal epithelial cell line and <jats:italic>Tabby</jats:italic> mouse skin tissue explants, Eda up‐regulated expression of <jats:styled-content style="fixed-case">ZO</jats:styled-content>‐1 and claudin‐1 through activation of the sonic hedgehog signalling pathway. We conclude that <jats:italic><jats:styled-content style="fixed-case">EDA</jats:styled-content></jats:italic> gene expression contributes to the maintenance of epithelial barrier function. Such insight may help efforts to identify novel strategies for improving management of <jats:styled-content style="fixed-case">XLHED</jats:styled-content> disease manifestations in a clinical setting.</jats:p> Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway Journal of Cellular and Molecular Medicine
spellingShingle Li, Sanming, Zhou, Jing, Zhang, Liying, Li, Juan, Yu, Jingwen, Ning, Ke, Qu, Yangluowa, He, Hui, Chen, Yongxiong, Reinach, Peter S., Liu, Chia‐Yang, Liu, Zuguo, Li, Wei, Journal of Cellular and Molecular Medicine, Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway, Cell Biology, Molecular Medicine
title Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway
title_full Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway
title_fullStr Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway
title_full_unstemmed Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway
title_short Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway
title_sort ectodysplasin a regulates epithelial barrier function through sonic hedgehog signalling pathway
title_unstemmed Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway
topic Cell Biology, Molecular Medicine
url http://dx.doi.org/10.1111/jcmm.13311