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COL4A4 belongs to the type IV collagen family. COL4A4 is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen. Defects in COL4A4 are a cause of Alport syndrome autosomal recessive (APSAR). Defects in COL4A4 are a cause of benign familial hematuria (BFH). This antibody is specific to COL4A4.