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Publication : Aortic Strain Correlates with Elastin Fragmentation in Fibrillin-1 Hypomorphic Mice.

First Author  Chen JZ Year  2019
Journal  Circ Rep Volume  1
Issue  5 Pages  199-205
PubMed ID  31123721 Mgi Jnum  J:299062
Mgi Id  MGI:6490113 Doi  10.1253/circrep.CR-18-0012
Citation  Chen JZ, et al. (2019) Aortic Strain Correlates with Elastin Fragmentation in Fibrillin-1 Hypomorphic Mice. Circ Rep 1(5):199-205
abstractText  Background: High frequency ultrasound has facilitated in vivo measurements of murine ascending aortas, allowing aortic strains to be gleaned from two-dimensional images. Thoracic aortic aneurysms associated with mutations in fibrillin-1 (FBN1) display elastin fragmentation, which may impact aortic strain. In this study, we determined the relationship between elastin fragmentation and aortic circumferential strain in wild type and fibrillin-1 hypomorphic (FBN1 (mgR/mgR)) mice. Methods and Results: Luminal diameters of the ascending aorta from wild type and FBN1 hypomorphic (FBN1 (mgR/mgR)) mice were measured in systole and diastole. Expansion of the ascending aorta during systole in male and female wild type mice was 0.21+/-0.02 mm (16.3%) and 0.21+/-0.01 mm (17.0%) respectively, while expansion in male and female FBN1 (mgR/mgR) mice was 0.11+/-0.04 mm (4.9%) and 0.07+/-0.02 mm (4.5%) respectively. Reduced circumferential strain was observed in FBN1 (mgR/mgR) mice compared to wild type littermates. Elastin fragmentation was inversely correlated to circumferential strain (R^2 = 0.628 p = 0.004) and significantly correlated with aortic diameter. (R^2 = 0.397, p = 0.038 in systole and R^2 = 0.515, p =0.013 in diastole). Conclusions: FBN1 (mgR/mgR) mice had increased aortic diameters, reduced circumferential strain, and increased elastin fragmentation. Elastin fragmentation in FBN1 (mgR/mgR) and their wild type littermates was correlated with reduced circumferential strain.
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