5nb1

Structural highlights

Disease

[CO4A4_HUMAN] Benign familial hematuria;Autosomal dominant Alport syndrome;Autosomal recessive Alport syndrome. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry.

Function

[CO4A4_HUMAN] Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen.