{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T11:33:00Z","timestamp":1791286380079,"version":"4.1.0"},"reference-count":40,"publisher":"SAGE Publications","issue":"6","license":[{"start":{"date-parts":[[2010,6,29]],"date-time":"2010-06-29T00:00:00Z","timestamp":1277769600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Vet Pathol"],"published-print":{"date-parts":[[2010,11]]},"abstract":"<jats:p>Vascular Ehlers-Danlos syndrome is a rare genetic disorder resulting from mutations in the \u03b1-1 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 diminishing wall strength of the aorta in these mice. Colons were grossly and histologically normal, but reduced strength and increased compliance of the wall were found in heterozygotes via pressure testing. Although mice demonstrated no life-threatening clinical signs or gross lesions of vascular subtype Ehlers-Danlos syndrome type IV, thorough histological examination of the aorta of heterozygous mice revealed the presence of a spectrum of lesions similar to those observed in human patients. Lesions increased in number and severity with age (0\/5 [0%] in 2-month-old males vs 9\/9 [100%] in 14-month-old males, P &lt; .05) and were more common in male than female mice (23\/26 [88.5%] vs 14\/30 [46.7%] in 9- to 21-month-old animals, P &lt; .05). Haploinsufficiency for Col3a1 in mice recapitulates features of vascular Ehlers-Danlos syndrome in humans and can be used as an experimental model.<\/jats:p>","DOI":"10.1177\/0300985810374842","type":"journal-article","created":{"date-parts":[[2010,6,29]],"date-time":"2010-06-29T22:10:20Z","timestamp":1277849420000},"page":"1028-1039","source":"Crossref","is-referenced-by-count":55,"title":["The Haploinsufficient\n                    <i>Col3a1<\/i>\n                    Mouse as a Model for Vascular Ehlers-Danlos Syndrome"],"prefix":"10.1177","volume":"47","author":[{"given":"T. K.","family":"Cooper","sequence":"first","affiliation":[{"name":"Department of Comparative Medicine, Penn State College of Medicine, Milton S. Hershey Medical Center, Hershey, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Q.","family":"Zhong","sequence":"additional","affiliation":[{"name":"Department of Comparative Medicine, Penn State College of Medicine, Milton S. Hershey Medical Center, Hershey, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Krawczyk","sequence":"additional","affiliation":[{"name":"Laboratory of Cardiovascular Science, National Institute on Aging, Baltimore, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H-J.","family":"Tae","sequence":"additional","affiliation":[{"name":"Laboratory of Cardiovascular Science, National Institute on Aging, Baltimore, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. A.","family":"M\u00fcller","sequence":"additional","affiliation":[{"name":"Laboratory of Cardiovascular Science, National Institute on Aging, Baltimore, Maryland"},{"name":"Present address: Department of Obstetrics and Gynecology, University of Leipzig, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Schubert","sequence":"additional","affiliation":[{"name":"Cardiovascular Physiology, Medical Faculty Mannheim, University of Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L. A.","family":"Myers","sequence":"additional","affiliation":[{"name":"Institute of Genetic Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. 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