Abstract
Vascular calcification is a complex and dynamic process occurring in various physiological conditions such as aging and exercise or in acquired metabolic disorders like diabetes or chronic renal insufficiency. Arterial calcifications are also observed in several genetic diseases revealing the important role of unbalanced or defective anti- or pro-calcifying factors. Pseudoxanthoma elasticum (PXE) is an inherited disease (OMIM 264800) characterized by elastic fiber fragmentation and calcification in various soft conjunctive tissues including the skin, eyes, and arterial media. The PXE disease results from mutations in the ABCC6 gene, encoding an ATP-binding cassette transporter primarily expressed in the liver, kidneys suggesting that it is a prototypic metabolic soft-tissue calcifying disease of genetic origin. The clinical expression of the PXE arterial disease is characterized by an increased risk for coronary (myocardial infarction), cerebral (aneurysm and stroke), and lower limb peripheral artery disease. However, the structural and functional changes in the arterial wall induced by PXE are still unexplained. The use of a recombinant mouse model inactivated for the Abcc6 gene is an important tool for the understanding of the PXE pathophysiology although the vascular impact in this model remains limited to date. Overlapping of the PXE phenotype with other inherited calcifying diseases could bring important informations to our comprehension of the PXE disease.
Highlights
ARTERIAL CALCIFICATION ischemic stroke (IS) AN INDEPENDENT RISK FACTOR OF CARDIOVASCULAR DISEASES Arterial calcification is gaining an increasing interest as an independent marker for cardiovascular (CV) diseases in acquired metabolic diseases, such as type II diabetes (Becker et al, 2008) and chronic renal insufficiency (Goodman et al, 2000)
Vascular calcification increases physiologically with age and studies from Egyptian mummies have revealed that arterial calcification is not a feature of modern life style due to the absence of risk factors such as smoking, high fat cholesterol diets encountered in these ancient civilizations (Allam et al, 2011)
Calcification of the intimal layers can complicate atherosclerotic plaques favoring the risk of rupture whereas deposit within the medial layer contributes to stiffen arterial wall leading to hypertension, cardiac hypertrophy, and heart failure (Demer and Tintut, 2008)
Summary
ARTERIAL CALCIFICATION IS AN INDEPENDENT RISK FACTOR OF CARDIOVASCULAR DISEASES Arterial calcification is gaining an increasing interest as an independent marker for cardiovascular (CV) diseases in acquired metabolic diseases, such as type II diabetes (Becker et al, 2008) and chronic renal insufficiency (Goodman et al, 2000). Three functional studies involving a small number (≈25) of PXE patients have focused on the carotid artery, an accessible large-sized musculo-elastic artery (Boutouyrie et al, 2001; Germain et al, 2003; Kornet et al, 2004).
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