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(Circulation. 2007;115:2687-2688.)
© 2007 American Heart Association, Inc.

Editorial

Through Thick and Thin Collagen Fibrils, Stress, and Aortic Rupture

Another Piece in the Jigsaw

Janet T. Powell, MD, PhD; Toste Länne, MD, PhD

From the Vascular Surgery Research Group, Imperial College at Charing Cross, London, UK (J.T.P.); and Department of Physiology and Cardiovascular Surgery, Faculty of Health Sciences, University Hospital, Linköping University, Linköping, Sweden (T.L.).

Correspondence to Janet T. Powell, MD, PhD, Vascular Surgery Research Group, Imperial College at Charing Cross, St Dunstan’s Rd, London W6 8RP, UK. E-mail j.powell@imperial.ac.uk

Key Words: Editorials • aorta • collagen • hemodynamics

An extract of the first 250 words of the full text is provided, because this article has no abstract.

This century has seen a leap forward in both treatment and understanding of the molecular and pathogenetic mechanisms underlying aortic dissection. The advantages of endovascular stenting are becoming obvious, and results from the Investigation of Stent Grafts in Patients With Type B Aortic Dissection (INSTEAD) trial are pending.¹ Molecular and pathogenetic advances include genetic defects mapped to several chromosomes in familial thoracic aortic dissection, including splice and missense mutations in the TGFBR2 gene on chromosome 3 associated with a Marfanoid syndrome.² Indeed, evidence is increasing that dysregulated transforming growth factor–ß signaling is an underlying mechanism in aneurysm formation. The small leucine-rich proteoglycans, including biglycan, bind transforming growth factor–ß and regulate collagen fibrillogenesis in vitro.³ The current article by Heegaard and colleagues,⁴ as well as the recent work of Takaluoma et al,⁵ uses mouse models to further our understanding of the weaknesses in collagen fibrils that can provoke the aortic dissection characteristic of Ehlers-Danlos syndrome.

Article p 2731

Heegaard et al⁴ elegantly demonstrate the altered morphology of aortic collagen fibrils in the absence of bgn, the biglycan core protein gene, which is encoded on the X chromosome in both mice and men. Absence of aortic biglycan leads to smaller collagen fibrils and a reduced resistance of the aorta to passive stress. In particular, the role of the adventitia in resisting passive stress is highlighted. The magnitude of these changes in fibril diameter and resistance to passive stress was much greater in males than in females, and only males were susceptible . . . [Full Text of this Article]