期刊
BIRTH DEFECTS RESEARCH PART A-CLINICAL AND MOLECULAR TERATOLOGY
卷 100, 期 9, 页码 712-720出版社
WILEY
DOI: 10.1002/bdra.23300
关键词
abdominal wall defect; neural tube defects; embryonic development; dermatan sulfate epimerase 1; epidermis
资金
- Cancerfonden [130388]
- Swedish Research Council [2012-2631, 2013-7478]
- Medical Faculty of Lund University
- Mizutani Foundation [120115]
- Albert Osterlund Foundation
- Greta and Johan Kock Foundation
- Anna-Greta Crafoord Foundation
Background: Dermatan sulfate (DS) is a highly sulfated polysaccharide with a variety of biological functions in extracellular matrix organization and processes such as tumorigenesis and wound healing. A distinct feature of DS is the presence of iduronic acid, produced by the two enzymes, DS-epimerase 1 and 2, which are encoded by Dse and Dsel, respectively. Methods: We have previously shown that Dse knockout (KO) mice in a mixed C57BL/6-129/SvJ background have an altered collagen matrix structure in skin. In the current work we studied Dse KO mice in a pure NFR genetic background. Results: Dse KO embryos and newborns had kinked tails and histological staining revealed significantly thicker epidermal layers in Dse KO mice when compared with heterozygote (Het) or wild-type (WT) littermates. Immunochemical analysis of the epidermal layers in newborn pups showed increased expression of keratin 5 in the basal layer and keratin 1 in the spinous layer. In addition, we observed an abdominal wall defect with herniated intestines in 16% of the Dse KO embryos. Other, less frequent, developmental defects were exencephaly and spina bifida. Conclusion: We conclude that the combination of defective collagen structure in the dermis and imbalanced keratinocyte maturation could be responsible for the observed developmental defects in Dse KO mice. In addition, we propose that Dse KO mice could be used as a model in pathogenetic studies of human fetal abdominal wall defects. (C) 2014 Wiley Periodicals, Inc.
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