Rabbit Polyclonal CollagenIVα5 Antibody

The collagen IV α5 antibody is a crucial tool in studying the structure and function of type IV collagen, a major component of basement membranes. Type IV collagen forms a hexameric network essential for tissue integrity, composed of six α chains (α1–α6). The α5 chain, encoded by the *COL4A5* gene (Xq22.3), pairs with α3 and α4 chains to create α3α4α5 heterotrimers, predominantly in the glomerular basement membrane (GBM), cochlea, and ocular tissues. Mutations in *COL4A5* cause X-linked Alport syndrome, characterized by progressive kidney dysfunction, sensorineural hearing loss, and ocular abnormalities. Antibodies targeting collagen IV α5 are used to detect its expression and distribution in tissues, aiding in diagnosing Alport syndrome and differentiating it from other glomerulopathies. In Alport patients, GBM staining with collagen IV α5 antibodies often reveals irregular or absent patterns due to pathogenic variants. These antibodies also facilitate research into disease mechanisms, such as basement membrane assembly and collagen chain interactions. Additionally, they help identify female carriers of X-linked Alport syndrome, who may exhibit mosaic α5 staining due to random X-chromosome inactivation. Beyond diagnostics, collagen IV α5 antibodies are employed in experimental models to study renal fibrosis, gene therapy outcomes, and basement membrane repair. Their specificity makes them valuable for immunohistochemistry, immunofluorescence, and Western blotting in both clinical and research settings.