Thrombosis Research
Volume 121, Issue 2 , Pages 159-162, 2007

Cystic fibrosis heterozygotes do not have increased platelet activation

  • Inge Tarnow

      Affiliations

    • Department of Physiology and Biochemistry, University of Copenhagen, The Faculty of Life Sciences, Frederiksberg C, Denmark
    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA, United States
  • ,
  • Alan D. Michelson

      Affiliations

    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA, United States
  • ,
  • Andrew L. Frelinger III

      Affiliations

    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA, United States
  • ,
  • Matthew D. Linden

      Affiliations

    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA, United States
  • ,
  • Youfu Li

      Affiliations

    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA, United States
  • ,
  • Marsha L. Fox

      Affiliations

    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA, United States
  • ,
  • Marc R. Barnard

      Affiliations

    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Center for Platelet Function Studies, University of Massachusetts Medical School, Worcester, MA, United States
  • ,
  • Brian P. O'Sullivan

      Affiliations

    • Department of Pediatrics, UMass Memorial Health Care and University of Massachusetts Medical School, Worcester, MA, United States
    • Corresponding Author InformationCorresponding author. Department of Pediatrics, UMass Memorial Health Care, 55 Lake Avenue, North, Worcester, MA 01655, United States. Tel.: +1 508 856 4155; fax: +1 508 856 2609.

Received 22 January 2007; received in revised form 10 April 2007; accepted 16 April 2007.

Abstract 

Introduction

We have previously demonstrated platelet hyperreactivity in cystic fibrosis (CF) patients. Carriers of one CF mutation (heterozygotes) have been shown to have abnormalities related to the presence of only one-half the normal amount of CF transmembrane conductance regulator protein. Platelet hyperreactivity in CF heterozygotes would be an important cardiovascular risk factor, since approximately 1 in 25 Caucasians is a CF carrier.

Materials and methods

We used highly sensitive assays of platelet activation to assess the difference between 16 CF heterozygotes and 16 age- and sex-matched healthy controls without CF mutations.

Results

We found no difference in platelet activation between CF heterozygotes and controls.

Conclusions

The 50% reduction in the CF transmembrane conductance regulator protein in heterozygotes is insufficient to cause platelet activation.

Abbreviations: CF, cystic fibrosis, CFTR, cystic fibrosis transmembrane conductance regulator, TRAP, thrombin receptor activating peptide, ADP, adenosine diphosphate, GP, glycoprotein, sCD40L, soluble CD40 ligand, TXB2, thromboxane B2, ANOVA, analysis of variance, PG, prostaglandin, MFI, mean fluorescence intensity.

Keywords: Platelets, Cystic fibrosis, Flow cytometry, Cystic fibrosis carriers, Platelet function

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PII: S0049-3848(07)00159-4

doi:10.1016/j.thromres.2007.04.004

Thrombosis Research
Volume 121, Issue 2 , Pages 159-162, 2007