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