目的筛选法洛氏四联症患儿血清差异表达蛋白,探索法洛氏四联症潜在的血清蛋白标志物。方法收集法洛氏四联症、室间隔缺损、健康人血清各20例,等量混合后使用多重亲和去除系统去除高丰度蛋白,经i TRAQ试剂标记后应用二维液相色谱分离,利用质谱技术对样品进行相对定量分析和生物信息学分析。结果筛选出置信度>95%的蛋白共292种,其中差异表达蛋白有37种,法洛氏四联症组较室间隔缺损组和健康组表达量均上调>1.3倍的蛋白有16种,下调<0.77倍的蛋白有21种。结论本研究应用同位素标记相对定量技术合MALDI-TOF/TOF MS筛选出包括半胱氨酸蛋白酶抑制剂C、玻连蛋白、胰岛素样生长因子2等在内的差异表达蛋白37种,这些蛋白可作为法洛氏四联症血清蛋白标志物的重要候选因子。 Objective To screen differentially expressed proteins in children with tetralogy of Fallot and explore the potential serum protein markers of tetralogy of Fallot. Methods Twenty cases of tetralogy of Fallot, ventricular septal defect and healthy human serum were collected. After the same amount of mixture was mixed, the multiple affinity removal system was used to remove the high abundance protein. After labeled with i TRAQ reagent, two-dimensional liquid chromatography The relative quantification and bioinformatics analysis of samples were carried out by mass spectrometry. Results A total of 292 proteins were screened with confidence> 95%, of which 37 proteins were differentially expressed. There were 16 proteins with a> 1.3-fold upregulation in patients with tetralogy of Fallot than those with ventricular septal defects and those with healthy controls. There are 21 proteins down-regulated <0.77 fold. Conclusion In this study, 37 kinds of differentially expressed proteins, including cystatin C, vitronectin and insulin-like growth factor 2, were screened by MALDI-TOF / TOF MS. As an important candidate for tetralogy of Fallot serum protein markers.