論文 - 詳細
RRC ID | 50370 |
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著者 | Hirose T, Suzuki I, Takahashi N, Fukada T, Tangkawattana P, Takehana K. |
タイトル | Morphometric analysis of cornea in the Slc39a13/Zip13-knockout mice. |
ジャーナル | J Vet Med Sci |
Abstract |
Ehlers-Danlos syndrome (EDS) is a group of hereditary diseases caused by mutation of extracellular matrix-related genes. Recently, spondylodysplastic EDS-Zip13 (spEDS-Zip13: OMIM 612350) was recognized as a new EDS type. This current study could reveal various morphometric differences of collagenous population in the proper substance of cornea between the wild type and spEDS-Zip13-knockout (Zip13-KO) mice. Blockade of Smad-signaling pathway might initiate these alterations. Predilected dissimilarity in level of transcriptional activity probably dictated morphology of keratocyte and shape and electron density of its nucleus. In addition, the imbalance of proteoglycans and glycosaminoglycans would also affect the diameter and arrangement of collagen fibrils. These findings would be considered as vulnerable characteristics of corneal stroma of the Zip13-KO mice. |
巻・号 | 80(5) |
ページ | 814-818 |
公開日 | 2018-5-18 |
DOI | 10.1292/jvms.18-0019 |
PMID | 29563392 |
PMC | PMC5989028 |
MeSH | Animals Cation Transport Proteins / genetics Cornea / pathology* Corneal Diseases / genetics* Corneal Diseases / pathology Corneal Keratocytes / pathology Ehlers-Danlos Syndrome / genetics* Ehlers-Danlos Syndrome / pathology Mice Mice, Inbred C57BL Mice, Knockout |
IF | 0.803 |
引用数 | 2 |
リソース情報 | |
実験動物マウス | RBRC06217 |