Abstract
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.
Highlights
Arterial remodeling refers to the myriad of structural and functional changes of the vascular wall that occur in response to disease, injury, or aging
Monogenetic diseases such as pseudoxanthoma elasticum (PXE), PXE-like syndrome, Marfan’s syndrome or Keutel syndrome are characterized by a clinical phenotype that is similar to that of arterial remodeling, but are caused by a specific defect that affects only one or several pathophysiological mechanisms of arterial remodeling
Single gene defects of these specific diseases affect major regulatory pathways such as vascular smooth muscle cells (VSMCs) phenotype switching, matrix degradation, and calcification that are involved in common cardiovascular disease and aging
Summary
Arterial remodeling refers to the myriad of structural and functional changes of the vascular wall that occur in response to disease, injury, or aging. Alterations in the activity of vitamin K-dependent proteins may affect the progression of vascular remodeling, including the induction of calcification Because of this complexity, it is difficult to study to what extent a single mechanism contributes to arterial remodeling. It is difficult to study to what extent a single mechanism contributes to arterial remodeling Monogenetic diseases such as pseudoxanthoma elasticum (PXE), PXE-like syndrome, Marfan’s syndrome or Keutel syndrome are characterized by a clinical phenotype that is similar to that of arterial remodeling, but are caused by a specific defect that affects only one or several pathophysiological mechanisms of arterial remodeling. Lessons learned from these relatively rare diseases may provide insight in more common, multifactorial cardiovascular diseases such as hypertension, diabetes mellitus, and chronic kidney disease as well as in normal vascular aging
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