尿常规肾脏疾病的实验诊断.ppt

今天汇报的内容主要有3部分 * 今天汇报的内容主要有3部分 * 今天汇报的内容主要有3部分 * 今天汇报的内容主要有3部分 * 今天汇报的内容主要有3部分 * 今天汇报的内容主要有3部分 * * * * * * * * * * * * 今天汇报的内容主要有3部分 * * * * * * * 今天汇报的内容主要有3部分 * 今天汇报的内容主要有3部分 * * 今天汇报的内容主要有3部分 * * * * * * * * * * * * * * * * 今天汇报的内容主要有3部分 * * * * In 1998,Kestil? et al. (23) isolated the gene mutated in congenital nephrotic syndrome of the Finnish type (CNF, NPHS1), a rare autosomal recessive disease characterized by massive nonselective proteinuria at birth and lack of a podocyte slit diaphragm. The disease gene was shown to encode a novel protein named “nephrin,” which in the kidney is solely located in glomerular podocytes. Nephrin was the first known protein to be localized, via immunoelectron microscopy, to the slit diaphragm (36). Inactivation of the gene in mice leads to massive proteinuria, absence of a slit diaphragm, and neonatal death Ann Med. 2006;38(7):483-92. Molecular basis of the glomerular filtration: nephrin and the emerging protein complex at the podocyte slit diaphragm. P?t?ri-Sampo A, Ihalmo P, Holth?fer H. Department of Bacteriology and Immunology, Haartman Institute, University of Helsinki, Helsinki, Finland. For more than three decades, the molecular composition of the interpodocyte slit diaphragm of the glomerular filtration barrier has remained elusive. The first electron microscopic studies described the slit diaphragm as a porous, zipper-like structure, but it was not until 1998 that the first transmembrane molecule of the slit diaphragm was identified: nephrin is a cell surface receptor of the immunoglobulin superfamily participating in cell-cell adhesion and signaling functions. Mutations in nephrin lead to the congenital nephrotic syndrome of the Finnish type, suggesting that nephrin is of pivotal importance for maintaining the filtration barrier. In recent years, the mapping of the genetic background of other inherited and acquired nephropathies and generation of transgenic animal models have led to a beginning of a new era in ne