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NDT Advance Access published online on April 2, 2008
Nephrology Dialysis Transplantation, doi:10.1093/ndt/gfn129
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© The Author [2008]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org


Understanding renal disorders as systemic diseases: the fascinating world of basement membranes beyond the glomerulus*

Oliver Gross

Department of Nephrology and Rheumatology, University of Göttingen, Göttingen, Germany

Correspondence and offprint requests to: Oliver Gross, Department of Nephrology and Rheumatology, University Hospital Göttingen, Robert-Koch Str. 40, D-37075 Göttingen, Germany. Tel: +49-551-39-6331; Fax: +49-551-39-8906; E-mail: gross.oliver@med.uni-goettingen.de

Keywords: Alport syndrome; chronic renal fibrosis; extracellular matrix; glomerular basement membrane; type IV collagen

The first 10% of the full text of this article appears below.

In the 27 December 2007 issue of the New England Journal of Medicine, Plaisier and colleagues [1] describe fascinating data on a new spectrum of autosomal dominant hereditary collagen diseases involving the COL4A1 gene. This gene codes for the {alpha}1-chain of type IV collagen, being the most important structural component of all basement membranes throughout the body. The paper was highly publicized and completes a trilogy of manuscripts about collagen type IV {alpha}1-chain mutations causing neonatal porencephaly, adult stroke, perinatal cerebral haemorrhage, skin lesions and nephropathies [1–3].

All basement membranes consist of a network of type IV collagen. Collagen IV monomers contain a long triple helical rod built by >1000 Gly-X-Y repeats, which is terminated at its C-terminal end by a globular NC1 domain, well known to nephrologists due to Goodpasture syndrome (Figure 1). In contrast to {alpha}. . . [Full Text of this Article]

(a) Can nephrologists’ knowledge about the glomerular basement membrane explain the clinical manifestations of COL4A1 mutations?
(b) Can nephrologists’ knowledge about the glomerular basement membrane predict the phenotype–genotype correlation in COL4A1-mutations?
(c) If nephrologists understand the glomerular matrix, will we understand the fascinating world beyond?

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