Abstract
Marfan syndrome (MFS) is associated with progressive aortic dilatation and endothelial dysfunction that lead to early acute dissection and rupture of the aorta and sudden death. Alteration in fatty acid (FA) metabolism can stimulate nitric oxide (NO) overproduction which increases the activity of the inducible form of NO synthase (iNOS) that is involved in endothelial dysfunction. We evaluated the participation of FA in the formation of thoracic aneurysms in MFS and its relation to the iNOS. Oleic acid (OA), iNOS, citrulline, nitrates and nitrites, TGF-β1, TNF-α, monounsaturated FA and NO synthase activity were significantly increased (p<0.05) in tissue from the aortas of MFS. Saturated FA, eNOS and HDL were significantly decreased (p<0.05). Arachidonic acid, delta-9 desaturase tended to increase and histological examination showed an increase in cystic necrosis, elastic fibers and collagen in MFS. The increase in OA contributes to the altered pathway of iNOS, which favors endothelial dysfunction and formation of the aortic aneurysms in MFS.
Keywords:
Aneurysm; Fatty acids; Inducible nitric oxide synthase; Marfan syndrome; Nitric oxide; Oleic acid.
Copyright © 2016 Elsevier Inc. All rights reserved.
MeSH terms
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Adult
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Aorta / metabolism*
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Aorta / pathology
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Aorta / surgery
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Aortic Aneurysm / metabolism*
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Aortic Aneurysm / pathology
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Aortic Aneurysm / surgery
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Arachidonic Acid / metabolism
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Case-Control Studies
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Cholesterol, HDL / metabolism
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Citrulline / metabolism
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Collagen / genetics
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Collagen / metabolism
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Female
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Gene Expression Regulation
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Humans
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Male
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Marfan Syndrome / metabolism*
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Marfan Syndrome / pathology
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Marfan Syndrome / surgery
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Middle Aged
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Nitrates / metabolism
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Nitric Oxide Synthase Type II / genetics
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Nitric Oxide Synthase Type II / metabolism
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Nitric Oxide Synthase Type III / genetics
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Nitric Oxide Synthase Type III / metabolism
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Nitrites / metabolism
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Oleic Acid / metabolism*
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Prospective Studies
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Signal Transduction
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Stearoyl-CoA Desaturase / genetics
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Stearoyl-CoA Desaturase / metabolism
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Transforming Growth Factor beta1 / genetics
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Transforming Growth Factor beta1 / metabolism
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Tumor Necrosis Factor-alpha / genetics
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Tumor Necrosis Factor-alpha / metabolism
Substances
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Cholesterol, HDL
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Nitrates
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Nitrites
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TGFB1 protein, human
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Transforming Growth Factor beta1
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Tumor Necrosis Factor-alpha
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Arachidonic Acid
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Citrulline
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Oleic Acid
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Collagen
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NOS2 protein, human
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NOS3 protein, human
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Nitric Oxide Synthase Type II
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Nitric Oxide Synthase Type III
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Stearoyl-CoA Desaturase