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. 2018 Jul 5;8:10219. doi: 10.1038/s41598-018-28545-0

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© 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/.

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Mitochondria damage in Ubiad1^N100S cornea. (A) Electron Microscopy showed mitochondrial degeneration in the corneal epithelial cells, stroma keratocytes, and endothelium of heterozygous and homozygous mice. Inset at lower right in each micrograph shows an individual mitochondrion at higher magnification. Mice were 4-month-old males. (B) Quantification of mitochondrial DNA copy number change in wild type mice and Ubiad1^N100S/N100S mice by real-time PCR. The fold change was 0.98 in wild type mice versus 1.27 in Ubiad1^N100S/N100S mice, P < 0.05. Mice were of mixed genders.