Ectodysplasin A regulates epithelial barrier function through sonic hedgehog signalling pathway
| Autor: | Yongxiong Chen, Chia-Yang Liu, Zuguo Liu, Sanming Li, Peter S. Reinach, Yangluowa Qu, Jing Zhou, Juan Li, Wei Li, Liying Zhang, Ke Ning, Jingwen Yu, Hui He |
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| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
Pathology medicine.medical_specialty X‐linked hypohidrotic ectodermal dysplasia Necrosis tight junction Mutant Ectodysplasin A medicine.disease_cause Epithelium Cornea 03 medical and health sciences 0302 clinical medicine medicine Animals Humans Hedgehog Proteins Hypohidrotic ectodermal dysplasia Sonic hedgehog Lung Skin Inflammation Mutation Tight Junction Proteins biology Tight junction epithelial barrier dysfunction Cell Biology Bacterial Infections Original Articles Ectodysplasins medicine.disease Hedgehog signaling pathway Recombinant Proteins Cell biology Mice Inbred C57BL 030104 developmental biology biology.protein Molecular Medicine Original Article medicine.symptom 030217 neurology & neurosurgery Signal Transduction |
| Zdroj: | Journal of Cellular and Molecular Medicine |
| ISSN: | 1582-4934 |
| Popis: | Ectodysplasin A (Eda), a member of the tumour necrosis factor superfamily, plays an important role in ectodermal organ development. An EDA mutation underlies the most common of ectodermal dysplasias, that is X‐linked hypohidrotic ectodermal dysplasia (XLHED) 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 ZO‐1 and claudin‐1 expression is largely reduced in epidermal, corneal and lung epithelia in Eda mutant Tabby 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 Tabby mouse skin tissue explants, Eda up‐regulated expression of ZO‐1 and claudin‐1 through activation of the sonic hedgehog signalling pathway. We conclude that EDA gene expression contributes to the maintenance of epithelial barrier function. Such insight may help efforts to identify novel strategies for improving management of XLHED disease manifestations in a clinical setting. |
| Databáze: | OpenAIRE |
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