| [Background] PAX2 (Paired Box2) gene encodes transcription factor that controls fetal developmental decisions and normally expresses in developmental stage of kidney. Ontogeny suggests that PAX2 is related with nephrogenesis and has a guidance for endothelial cells and mesangial cells. Deletion or mutation of Pax2 gene may give rise to anephrogenesis and renal dysplasia. Recently, some reporters showed that PAX2 took part in pathogenesis of renal disease in humans. The phenotypic alteration of podocytes was found in idiopathic collapsing glomerulopathy and HIV-associated nephropathy suggesting that re-expression of Pax2 in podocytes was one of the important change for the development of cell lesions. The study on renal histological features with PAX2 gene mutation revealed that abnormal expression of PAX2 was related with interstitial fibrosis, tubular atrophy. At present, it's uncertain for the control mechanism and target genes, as well as its function. FGF-FGFR4 Signal, fibroblast growth factor (FGF) and its receptor (FGFR4) may regulate expression of PAX2 having action of suppressing apoptosis, facilitating proliferation and differentiation. C-myc, one gene regulating apoptosis making cell proliferate together with other genes of proliferation, not only is related with kidney development but also inactivated in mature kidney, may be target gene of PAX2. There is little previous studies for PAX2 in fetal kidney of humans, and most of the gestation age studied were below 28wks gestation age, hence which could not fully represent the pattern of PAX2 expression in humans. The investigation for renal disease is much less and could not really reflect feature of PAX2 expression in renal disease. Therefore, it will bring new light into mechanism of renal pathogenesis to observe PAX2 expression systemically on fetal kidney and several renal diseases in childhood and research on possible role in renal pathogenesis.[Objectives] To evaluate the expression of PAX2 in developing kidney withvarious gestation ages in order to show the pattern of PAX2 expression and understand the function of PAX2 in humans, as well as to offer the basis for study on the link between the expression pattern and renal disease in childhood. To investigate the PAX2 expression in-i,renal diseases in childhood and try to find the role of PAX2 in the mechanism of these conditions, so as to bring new insight into the study for the mechanism of renal pathogenesis and provide evidence of gene interference to renal disease in the future.[Methods] To investigate the expression of PAX2, C-myc and FGFR4 innormal fetal kidney from 8 to 34 weeks gestation age, as well as primary nephroticsyndrome (PNS), acute glomerulonephritis (AGN), Henoch-Schoenlein purpura nephritis (HSPN), isolated hematuria (IHU) in childhood by immumohistochemistry and in situ hybridization, and to make a correlation analysis on pathology integral and function of kidney with expression of PAX2 , C-myc and FGFR4.[Results] 1. PAX2 expression was mainly in the nephrogenesis zone,evidently decreased toward the center of mature nephrons. The locus of PAX2 expression was related to the stage of glomeruli, not to the gestation age of fetus.The intense immunostaining of PAX2 was showed in ureteric bud tip, condensing mesenchyme, renal vesicle and comma-shaped body, the primitive tubules of S-shaped body stained evidently. Individual difference of PAX2 expression occurred in the primitive tubules in S-shapedbody and immature visceral epithelial cells (podocytes) of glomeruli in C-stage, as well as in parietal epithelial cells, where some cells showed no expression, yet others powerful expression. Attenuation of PAX2 was detected in tubules in later stage of renal development, but a few of distal tubules and collecting tubules expressing continuously.2. PAX2 re-expression is showed in all sections of various renal diseases, more intense staining in distal tubules than in proximal tubules, podocytes paintly, and...
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