摘要
目的 :检测肾病综合征 (NS)患儿肾小球足细胞中nephrin、podocin、α actinin及WT1的表达和分布特征 ,以探讨这些分子在蛋白尿发生中的可能作用。 方法 :用免疫荧光染色 ,激光扫描共聚焦显微镜采集图像及图像分析的方法 ,检测 2 5例NS患儿 [其中肾脏病理为微小病变 (MCD) 2例 ,系膜增生性肾小球肾炎 (MsPGN) 17例 ,局灶节段性肾小球硬化 (FSGS) 5例 ,新月体性肾小球肾炎 (CREGN) 1例 ]、9例单纯性血尿患儿及 9例对照肾组织中nephrin、podocin、α actinin及WT1的表达。 结果 :(1)NS患儿肾小球中podocin的表达量 (82 9± 2 1 5 )与单纯性血尿组 (10 3 6± 2 0 2 )及对照组 (110 8± 15 4 )比较差异显著 (P =0 0 12 ,P =0 0 0 1) ;WT1的表达量 (6 1 3± 9 2 3)与对照组 (6 9 7± 9 2 7)比较差异也显著 (P =0 0 37) ;nephrin和α actinin表达量 (12 9 3± 2 2 6 ,10 4 3± 19 0 )与单纯性血尿组(132 1± 7 4 0 ,94 9± 13 0 )及对照组 (133 9± 8 5 ,10 3 6± 15 0 )比较差异均不显著 (P >0 0 5 )。 (2 )对照组肾组织中nephrin和podocin的染色均见于肾小球毛细血管壁 ,呈均匀、线状分布 ;α actinin主要沿肾小球毛细血管壁呈点状分布 ;WT1的染色主要集中于肾小球细胞核。 (3)
Objective:To investigate the possible roles of nephrin,podocin,α-actinin and WT1 in the development of proteinuria in children with nephrotic syndrome. Methodology:A total of 43 renal biopsies were enrolled in this study.They were 25 cases from the children with primary nephrotic syndrome,9 from the children with isolated hematuria,and 9 from nephrectomy kidneys as control group.Immunofluorescence staining and confocal laser scanning microscopy were used to detect the expressions and distributions of nephrin,podocin,α-actinin and WT1 molecules on glomeruli. Results:① In the children with nephrotic syndrome,the glomerular expression of podocin(82.9±21.5) decreased significantly compared with that in hematuria (103.6±20.2,P=0.012) and control groups (110.8±15.4,P=0.001).② There were no significant differences in the expressions of nephrin and α-actinin among nephrotic syndrome (129.3±22.6,104.3±19.0),hematuria (132.1±7.40,94.9±13.0) and control groups (133.9±8.50,103.6±15.0,P>0.05).③ In control group,the distributions of nephrin and podocin showed a linear pattern along the glomerular basement membrane,α-actinin staining showed a dotted line pattern,and the staining of WT1 was mainly restricted to the nucleus.④ The podocin expression in nephrotic syndrome children with mesangial proliferative glomerulonephritis (88.0±22.1) and focal segmental glomerulosclerosis (66.4±4.59) decreased significantly compared with that in control group (P=0.01,P<0.05,respectively).The distribution patterns of nephrin,podocin and α-actinin also showed differences between the controls and nephrotic syndrome children.The staining of nephrin and podocin on glomeruli were uneven and nonlinear,and the distribution of α-actinin was linear on some part of capillary walls. Conclusion:A dramatic decrease of glomerular podocin expression was found in children with nephrotic syndrome.The abnormal distribution of nephrin,podocin and α-actinin in nephrotic syndrome was also revealed.It suggested that these molecules mig
出处
《肾脏病与透析肾移植杂志》
CAS
CSCD
2002年第3期205-210,共6页
Chinese Journal of Nephrology,Dialysis & Transplantation
基金
国家自然科学基金资助项目 ( 30 170 992 )
