Nephrotic mice (ICGN strain): a model of diffuse mesangial sclerosis in infantile nephrotic syndrome.

摘要: The ICGN mouse strain is a unique model for naturally occurring nephrotic syndrome. In the present study, we examined onset of clinical manifestation syndrome and determined sequence intraglomerular events associated with progression conditions. Laboratory analysis revealed that homozygous (nep/nep) mice showed urinary albumin excretion during suckling stage, rapidly leading to hypoalbuminemia accompanied by body growth failure. Renal pathology demonstrated an initial event in was observed 3 weeks after birth form mesangiolytic lesions, characterized microaneurysm, platelet accumulation capillary ballooning. 6-week-old mice, mesangial sclerosis, expansion glomerular hypertrophy, diffuse fashion. Immunohistochemistry cells 3-week-old were positive α-smooth muscle actin, suggesting phenotypic change cells. Mesangial expansion, confirmed over-deposition type I collagen, evident until 6 weaning, while it interest fibrogenic cytokines such as platelet-derived factor transforming factor-β not detected sclerotic glomeruli throughout observations. Furthermore, features shown be resistant steroid therapy high dose prednisolone. Our results suggest hereditary disease, may genetically generated through early myofibroblast formation, which occurs develops probably independently up-regulation these cytokines. conclusion, (ICGN strain) believed good investigating only conditions but also cellular molecular pathogenesis sclerosis steroid-resistant infantile