Literature DB >> 20926894

Marfan syndrome decreases Ca2+ wave frequency and vasoconstriction in murine mesenteric resistance arteries without changing underlying mechanisms.

H T Syyong1, A W Y Chung, C van Breemen.   

Abstract

BACKGROUND/AIMS: Vascular smooth muscle in Marfan syndrome, a connective tissue disorder caused by mutations in FBN1 encoding fibrillin-1, is associated with decreased tonic contraction. As Ca(2+) waves are tightly associated with vasoconstriction, we hypothesized decreased tonic contraction in Marfan syndrome is due to aberrant Ca(2+) wave signaling.
METHODS: Isometric force and intracellular Ca(2+) were measured from second-order mesenteric arteries from mice heterozygous for the Fbn1 allele encoding a cysteine substitution (Fbn1(C1039G/+)).
RESULTS: Phenylephrine concentration-dependently induced tonic contraction associated with sustained repetitive oscillations in intracellular [Ca(2+)] in both control and Marfan vessels, although Marfan vessels displayed significantly decreased Ca(2+) wave frequency and decreased number of cells exhibiting waves. Inhibition of sarcoplasmic reticulum Ca(2+) re-uptake by cyclopiazonic acid abolished Ca(2+) waves, dramatically decreasing tonic contraction. Nifedipine significantly reduced Ca(2+) wave frequency and tonic contraction, while the nifedipine-insensitive component was abolished by SKF-96365. Ca(2+) waves and tonic contraction were abolished by 2-aminoethoxydiphenylborate, but were unaffected by ryanodine or tetracaine.
CONCLUSION: Phenylephrine-induced Ca(2+) waves underlie tonic contraction in resistance-sized mesenteric arteries and appear to be produced by repetitive cycles of regenerative Ca(2+) release from the sarcoplasmic reticulum. Decreased frequency of Ca(2+) waves in Marfan syndrome appears to be responsible for reduced tonic contraction.
Copyright © 2010 S. Karger AG, Basel.

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Year:  2010        PMID: 20926894     DOI: 10.1159/000318804

Source DB:  PubMed          Journal:  J Vasc Res        ISSN: 1018-1172            Impact factor:   1.934


  3 in total

1.  An iPSC-derived vascular model of Marfan syndrome identifies key mediators of smooth muscle cell death.

Authors:  Alessandra Granata; Felipe Serrano; William George Bernard; Madeline McNamara; Lucinda Low; Priya Sastry; Sanjay Sinha
Journal:  Nat Genet       Date:  2016-11-28       Impact factor: 38.330

Review 2.  Vascular smooth muscle cells in Marfan syndrome aneurysm: the broken bricks in the aortic wall.

Authors:  Gianluca L Perrucci; Erica Rurali; Aoife Gowran; Alessandro Pini; Carlo Antona; Roberto Chiesa; Giulio Pompilio; Patrizia Nigro
Journal:  Cell Mol Life Sci       Date:  2016-08-17       Impact factor: 9.261

3.  Characterization of the thoracodorsal artery: morphology and reactivity.

Authors:  Marie Billaud; Alexander W Lohman; Adam C Straub; Thibaud Parpaite; Scott R Johnstone; Brant E Isakson
Journal:  Microcirculation       Date:  2012-05       Impact factor: 2.628

  3 in total

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