Review ARTICLE

Front. Genet., 13 December 2012 | http://dx.doi.org/10.3389/fgene.2012.00290

Mechanisms of arterial remodeling: lessons from genetic diseases

  • 1Department of Internal Medicine, Medical Centre and Cardiovascular Research Institute Maastricht, Maastricht University, Maastricht, Netherlands
  • 2Department of Biochemistry, Cardiovascular Research Institute Maastricht, Maastricht University, Maastricht, Netherlands

Vascular disease is still the leading cause of morbidity and mortality in the Western world, and the primary cause of myocardial infarction, stroke, and ischemia. The biology of vascular disease is complex and still poorly understood in terms of causes and consequences. Vascular function is determined by structural and functional properties of the arterial vascular wall. Arterial stiffness, that is a pathological alteration of the vascular wall, ultimately results in target-organ damage and increased mortality. Arterial remodeling is accelerated under conditions that adversely affect the balance between arterial function and structure such as hypertension, atherosclerosis, diabetes mellitus, chronic kidney disease, inflammatory disease, lifestyle aspects (smoking), drugs (vitamin K antagonists), and genetic abnormalities [e.g., pseudoxanthoma elasticum (PXE), Marfan's disease]. The aim of this review is to provide an overview of the complex mechanisms and different factors that underlie arterial remodeling, learning from single gene defect diseases like PXE, and PXE-like, Marfan's disease and Keutel syndrome in vascular remodeling.

Keywords: arterial remodeling, calcification, genetic disease, vitamin K, vitamin K-antagonists

Citation: van Varik BJ, Rennenberg RJMW, Reutelingsperger CP, Kroon AA, de Leeuw PW and Schurgers LJ (2012) Mechanisms of arterial remodeling: lessons from genetic diseases. Front. Gene. 3:290. doi: 10.3389/fgene.2012.00290

Received: 15 October 2012; Accepted: 23 November 2012;
Published online: 13 December 2012.

Edited by:

Olivier M. Vanakker, Ghent University Hospital, Belgium

Reviewed by:

Ying Xu, West Virginia University, USA
Wei Zhang, University of Michigan, USA

Copyright © 2012 van Varik, Rennenberg, Reutelingsperger, Kroon, de Leeuw and Schurgers. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in other forums, provided the original authors and source are credited and subject to any copyright notices concerning any third-party graphics etc.

*Correspondence: Leon J. Schurgers, Department of Biochemistry, Cardiovascular Research Institute Maastricht, Maastricht University, Universiteitssingel 50, PO Box 616, 6200 MD Maastricht, Netherlands. e-mail: l.schurgers@maastrichtuniversity.nl