Morphometric analysis of thoracic aorta in Slc39a13/Zip13-KO mice View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2019-04

AUTHORS

Takuya Hirose, Takamasa Shimazaki, Naoki Takahashi, Toshiyuki Fukada, Takafumi Watanabe, Prasarn Tangkawattana, Kazushige Takehana

ABSTRACT

Ehlers-Danlos syndrome (EDS) is a collection of inheritable diseases involving the musculoskeletal, integumentary and visual systems. Spondylodysplastic EDS-ZIP13 (spEDS-ZIP13: OMIM 612350) was recently defined as a new form of EDS. Although vasculitis has been found in many spEDS-ZIP13 patients, vascular pathology has not been included as a pathognomonic lesion of this type of EDS. We investigate the morphometry of the thoracic aorta in wild-type and Zip13-knockout (Zip13-KO) mice. Our assessment found abnormalities in the number and morphology of elastic and cellular components in the aortic wall, especially the tunica media, of Zip13-KO mice, indicating aortic fragility. Accordingly, our major findings (vascular smooth muscle cells with small nuclei, small percentage of elastic membrane area per tunica media, many large elastic flaps) should be considered vulnerable characteristics indicating fragility of the aorta in patients with spEDS-ZIP13. More... »

PAGES

1-5

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s00441-018-2977-9

DOI

http://dx.doi.org/10.1007/s00441-018-2977-9

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1111099750

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/30610452


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