Mechanical and pharmacological approaches to investigate the pathogenesis of Marfan syndrome in the abdominal aorta

Journal of Vascular Research
Ada W Y ChungCornelis van Breemen

Abstract

Occurrence of disease complications in the abdominal aorta in Marfan syndrome, a connective tissue disorder caused by mutations in the gene encoding fibrillin-1, is relatively rare. We hypothesized that Marfan syndrome could affect the structure, vasomotor function and mechanical property of the abdominal aorta. Abdominal aorta from mice at 3, 6, 9 and 12 months of age, heterozygous for the Fbn1 allele encoding a cysteine substitution (Fbn1(C1039G/+), Marfan mice, n = 50), were compared with those from age-matched control littermates (n = 50). Marfan abdominal aorta demonstrated pronounced elastic fiber degradation and disorganization, concomitant with an increased aortic stiffness during aging. In the isometric force measurement, vasoconstriction in response to membrane depolarization or phenylephrine stimulation was similar in both Marfan and control abdominal aorta. However, Marfan abdominal aorta was less sensitive to the inhibition of the phenylephrine-induced contraction by indomethacin and SQ-29548, during which the release of thromboxane A(2) was one half of that of the controls. Nevertheless, the protein expression of cyclooxygenase-1 and cyclooxygenase-2 detected by Western immunoblotting was not different between the...Continue Reading

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Citations

Dec 15, 2010·European Heart Journal·Lea MimounGuillaume Jondeau
Oct 24, 2009·Arteriosclerosis, Thrombosis, and Vascular Biology·Luca CartaFrancesco Ramirez
Apr 20, 2010·Arthritis Research & Therapy·Emma C Derrett-SmithChristopher P Denton
Mar 25, 2011·The Journal of Pathology·Boubacar MarikoGilles Faury
Dec 24, 2010·The Journal of Pharmacology and Experimental Therapeutics·Mitra EsfandiareiCornelis van Breemen
Oct 10, 2009·British Journal of Pharmacology·H H Clarice YangAda W Y Chung

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