Cystic fibrosis heterozygotes do not have increased platelet activation |
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Authors: | Tarnow Inge Michelson Alan D Frelinger Andrew L Linden Matthew D Li Youfu Fox Marsha L Barnard Marc R O'Sullivan Brian P |
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Affiliation: | Department of Physiology and Biochemistry, University of Copenhagen, The Faculty of Life Sciences, Frederiksberg C, Denmark. |
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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. |
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Keywords: | 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. |
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