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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 α1-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 α1-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 α-chains of the fibre forming collagens (types I, II, III and V), collagen IV chains contain interruptions in their Gly-X-Y repeats. These interruptions endow the collagen IV triple helix with a high degree of flexibility compared to fibre-forming collagens. Six genetically distinct α-chains of type IV collagen have been identified, forming a network of α1α1α2(IV), α3α4α5(IV) and α5α5α6(IV) [4]. Whereas the α1α1α2(IV) trimers are widely expressed in all basement membranes throughout the body, the α3α4α5(IV) network localizes to the glomerular basement membrane, lung, eye and lens and the α5α5α6(IV) network to Bowman’s capsule and the oesophagus [5]. Alport syndrome is derived from a mutation of either the COL4A3, COL4A4 or COL4A5 gene, coding for α3, α4 or α5 chain of type IV collagen, i.e. collagen types that constitute basement membranes in the renal glomerulus, the

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