Cystic fibrosis heterozygotes do not have increased platelet activation
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
© 2007 Elsevier Ltd. All rights reserved.
