RRC ID 54385
Author Hirose T, Shimazaki T, Takahashi N, Fukada T, Watanabe T, Tangkawattana P, Takehana K.
Title Morphometric analysis of thoracic aorta in Slc39a13/Zip13-KO mice.
Journal Cell Tissue Res
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.
Volume 376(1)
Pages 137-141
Published 2019-4-1
DOI 10.1007/s00441-018-2977-9
PII 10.1007/s00441-018-2977-9
PMID 30610452
MeSH Animals Aorta, Thoracic / abnormalities* Aorta, Thoracic / pathology Cation Transport Proteins / genetics Ehlers-Danlos Syndrome / pathology* Elasticity Mice Mice, Inbred C57BL Mice, Knockout Muscle, Smooth, Vascular / abnormalities* Muscle, Smooth, Vascular / ultrastructure Osteochondrodysplasias / pathology*
IF 4.044
Times Cited 0
Mice RBRC06217