Type IV collagen is the major structural component of glomerular basement membranes forming a "chicken-wire" meshwork together with laminins, proteoglycans and entactin/nidogen. There are six type IV collagen isoforms, alpha 1(IV)-alpha 6(IV), each of which can form a triple helix structure with 2 other chains to generate type IV collagen network. Defects in type IV collagen are a cause of Alport syndrome autosomal recessive (APSAR), characterized by glomerulonephritis, renal failure, sensorineural deafness and eye abnormalities. Defects in type IV collagen are also a cause of benign familial hematuria (BFH). The antibody is directed against rodent alpha 1 isoform.