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Progressive kidney failure is a genetically and clinically heterogeneous group of disorders. Podocyte foot processes and the interposed glomerular slit diaphragm are essential components of the permeability barrier in the kidney. Mutations in genes encoding structural proteins of the podocyte lead to the development of proteinuria, resulting in progressive kidney failure and focal segmental glomerulosclerosis. Here, we show that the canonical transient receptor potential 6 (TRPC6) ion channel is expressed in podocytes and is a component of the glomerular slit diaphragm. We identified five families with autosomal dominant focal segmental glomerulosclerosis in which disease segregated with mutations in the gene TRPC6 on chromosome 11q. Two of the TRPC6 mutants had increased current amplitudes. These data show that TRPC6 channel activity at the slit diaphragm is essential for proper regulation of podocyte structure and function.  相似文献   
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用红外光谱法测定表明:各种硅胶负载仲进型铑催化剂上,线CO/桥CO红外吸收强度比值与金属助剂(M)的关系变化顺序为(50:1)(Rh-Mn)〉(22:1)(Rh-Li)〉(9.1:1)(Rh-Mn-Fe-Li)〉(3.5:1)(Rh-Fe)〉2.8:1)(Rh)。在各种Rh-M/SiO2催化剂上,线、桥CO吸附态的加氢原位FTIR跟踪实验表明:随着加氢的进行,线CO/桥CO吸收强度比值均逐渐减少…  相似文献   
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