The Haploinsufficient Col3a1 Mouse as a Model for Vascular Ehlers-Danlos Syndrome
Abstract
Vascular Ehlers-Danlos syndrome is a rare genetic disorder resulting from mutations in the α-1 chain of type III collagen (COL3A1) and manifesting as tissue fragility with spontaneous rupture of the bowel, gravid uterus, or large or medium arteries. The heterozygous Col3a1 knockout mouse was investigated as a model for this disease. The collagen content in the abdominal aorta of heterozygotes was reduced, and functional testing revealed...
Paper Details
Title
The Haploinsufficient Col3a1 Mouse as a Model for Vascular Ehlers-Danlos Syndrome
Published Date
Jun 29, 2010
Journal
Volume
47
Issue
6
Pages
1028 - 1039
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