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Embryonic Surface Ectoderm-specific Mitofusin 2 Conditional Knockout Induces Congenital Cataracts in Mice

Inherited mitochondrial mutations can result in mitochondrial dysfunction or stochastic oxidative damage. Cumulative mitochondrial damage is an important factor in age-related disorders, such as cataracts and macular degeneration. Mfn2 mediates the fusion of mitochondria and contribute to the dynami...

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Autores principales: Zhao, Jiangyue, Wu, Xinwei, Wu, Danhong, Yu, Yinhui, Yu, Yibo, Wang, Yao, Fu, Qiuli, Zhang, Jinsong, Yao, Ke
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5784114/
https://www.ncbi.nlm.nih.gov/pubmed/29367651
http://dx.doi.org/10.1038/s41598-018-19849-2
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author Zhao, Jiangyue
Wu, Xinwei
Wu, Danhong
Yu, Yinhui
Yu, Yibo
Wang, Yao
Fu, Qiuli
Zhang, Jinsong
Yao, Ke
author_facet Zhao, Jiangyue
Wu, Xinwei
Wu, Danhong
Yu, Yinhui
Yu, Yibo
Wang, Yao
Fu, Qiuli
Zhang, Jinsong
Yao, Ke
author_sort Zhao, Jiangyue
collection PubMed
description Inherited mitochondrial mutations can result in mitochondrial dysfunction or stochastic oxidative damage. Cumulative mitochondrial damage is an important factor in age-related disorders, such as cataracts and macular degeneration. Mfn2 mediates the fusion of mitochondria and contribute to the dynamic balance between fusion and fission that determines mitochondria morphology. We report here that conditional loss of Mfn2 function in the head surface ectoderm leads to a range of congenital eye defects, including small, opacified lens and small eyeball in the most severe phenotypes. The Le-Cre transgenic mouse line and Mfn2 flox mouse line were used in this study to generate Mfn2 conditional knockout mice. Our study revealed Mfn2 gene function in lens development and addressed the relationship between the mitochondria and lens transparency. Conditional loss of Mfn2 affected lens epithelium cell proliferation, apoptosis and ultrastructure of mitochondria. We conclude that proper development of the lens and lens transparency depend on normal Mfn2 gene function.
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spelling pubmed-57841142018-02-07 Embryonic Surface Ectoderm-specific Mitofusin 2 Conditional Knockout Induces Congenital Cataracts in Mice Zhao, Jiangyue Wu, Xinwei Wu, Danhong Yu, Yinhui Yu, Yibo Wang, Yao Fu, Qiuli Zhang, Jinsong Yao, Ke Sci Rep Article Inherited mitochondrial mutations can result in mitochondrial dysfunction or stochastic oxidative damage. Cumulative mitochondrial damage is an important factor in age-related disorders, such as cataracts and macular degeneration. Mfn2 mediates the fusion of mitochondria and contribute to the dynamic balance between fusion and fission that determines mitochondria morphology. We report here that conditional loss of Mfn2 function in the head surface ectoderm leads to a range of congenital eye defects, including small, opacified lens and small eyeball in the most severe phenotypes. The Le-Cre transgenic mouse line and Mfn2 flox mouse line were used in this study to generate Mfn2 conditional knockout mice. Our study revealed Mfn2 gene function in lens development and addressed the relationship between the mitochondria and lens transparency. Conditional loss of Mfn2 affected lens epithelium cell proliferation, apoptosis and ultrastructure of mitochondria. We conclude that proper development of the lens and lens transparency depend on normal Mfn2 gene function. Nature Publishing Group UK 2018-01-24 /pmc/articles/PMC5784114/ /pubmed/29367651 http://dx.doi.org/10.1038/s41598-018-19849-2 Text en © The Author(s) 2018 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.
spellingShingle Article
Zhao, Jiangyue
Wu, Xinwei
Wu, Danhong
Yu, Yinhui
Yu, Yibo
Wang, Yao
Fu, Qiuli
Zhang, Jinsong
Yao, Ke
Embryonic Surface Ectoderm-specific Mitofusin 2 Conditional Knockout Induces Congenital Cataracts in Mice
title Embryonic Surface Ectoderm-specific Mitofusin 2 Conditional Knockout Induces Congenital Cataracts in Mice
title_full Embryonic Surface Ectoderm-specific Mitofusin 2 Conditional Knockout Induces Congenital Cataracts in Mice
title_fullStr Embryonic Surface Ectoderm-specific Mitofusin 2 Conditional Knockout Induces Congenital Cataracts in Mice
title_full_unstemmed Embryonic Surface Ectoderm-specific Mitofusin 2 Conditional Knockout Induces Congenital Cataracts in Mice
title_short Embryonic Surface Ectoderm-specific Mitofusin 2 Conditional Knockout Induces Congenital Cataracts in Mice
title_sort embryonic surface ectoderm-specific mitofusin 2 conditional knockout induces congenital cataracts in mice
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5784114/
https://www.ncbi.nlm.nih.gov/pubmed/29367651
http://dx.doi.org/10.1038/s41598-018-19849-2
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