ABSTRACT
Alport syndrome is a rare genetic disorder of specialized basement membranes in the kidney,ear,and eye.Eighty percent of patients have X-linked disease caused by mutations in the COL4A5 gene;autosomal forms of the other Alport syndrome are caused by mutations in COI4A3 and COL4A4 genes.These mutations result in progressive irregular thickening,thinning,and splitting of the GBM,leading to end stage renal failure.During the past two decades,research into this rare disease has focused on the effects of mutations in collagen type Ⅳ α chains and the role of changes in podocytes and the glomerular basement membrane that lead to early kidney fibrosis.In this review,we discuss the latest basic and clinical research on Alport syndrome,focusing on the roles of podocyte pathology and the extracellular matrix.
ABSTRACT
Aim To observe the thickness changes of cardiomyocyte and microvascular basement membranes(BM)and the expression of collagen Ⅳ in Gk rats of different months and STZ-induced diabetic rats,and to explore the method that operated easily and could assess the thickness of myocardial BM.Methods GK rats of the fourth,fifth,sixth,seventh months,Streptozotocin induced diabetes rats of fifth months and normal Wister rats were respectively divided into different groups with ev eight rats in each group.The myocardial section was stained by methenamine silver and immunohistochemistry was performed for collagen Ⅳ.Left ventricular endocardium was selected as measuring view.Through computer image analysis,the ratio of BM positive staining density(BMPSD)and positive staining density of collagen Ⅳ(CPSD)were calculated separately in all myocardium.Take the ratio to assess the thickness of myocardial BM and the expression of collagen Ⅳ.Results The glycemia,BMPSD and CPSD of the two diabetes rats were higher than those of the normal rats,having highly remarkable difference(P0.05).Conclusion By methenamine silver staining for myocardial section,calculating BMPSD through computer image analysis,can be an index to assess the thickness of myocardial BM.