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Previous Volume 354:1387-1401 March 30, 2006 Number 13 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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The inherited forms of proteinuria comprise a heterogeneous group of rare renal diseases in which glomerular dysfunction and proteinuria are prominent. Despite the rarity of hereditary proteinuria syndromes, genetic, biochemical, and structural studies of these diseases have made important contributions to our knowledge of how the normal glomerular filter works and the mechanisms of proteinuria.

The courses of these diseases can vary. Some patients present with severe proteinuria and congenital nephrotic syndrome, whereas others have only moderate proteinuria and focal segmental glomerulosclerosis. Regardless of its cause, the disease often progresses to end-stage renal disease. Classification of these syndromes has been . . . [Full Text of this Article]

The Glomerular Filtration Barrier

Fenestrated Endothelium

Glomerular Basement Membrane

The Podocyte Slit Diaphragm

            Nephrin

            Neph1 and Neph2

            FAT1 and FAT2

            Podocin

            CD2AP

            Other Protein Constituents of the Slit Diaphragm

            Structure of the Slit Diaphragm

Hereditary Proteinuria Syndromes

            CNF

Corticosteroid-Resistant Nephrotic Syndrome

Pierson's Syndrome

Nail–Patella Syndrome

Denys–Drash Syndrome and Frasier's Syndrome

Autosomal Dominant Focal Segmental Glomerulosclerosis

Conclusions

Source Information

From the Department of Medical Biochemistry and Biophysics, Karolinska Institute, Stockholm (K.T., J.P.); and the Electron Microscopy Unit, Institute of Biotechnology, University of Helsinki, Helsinki (J.W.).

Address reprint requests to Dr. Tryggvason at the Department of Medical Biochemistry and Biophysics, Karolinska Institute, 171 77 Stockholm, Sweden, or at karl.tryggvason@ki.se.

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