Collagen Type IV Alpha 3 (COL4A3) Antibody

Background

COL4A3 is one of the six α-chains that compose type IV collagen, a major structural component of basement membranes. COL4A3 assembles into a triple-helical network with COL4A4 and COL4A5 to provide mechanical support, structural stability, and selective permeability in tissues such as the kidney glomerulus, lung alveoli, and the ocular lens. Mutations in COL4A3 are associated with Alport syndrome, a hereditary disorder characterized by progressive kidney disease, sensorineural hearing loss, and ocular abnormalities caused by defective basement membranes. It also plays a role in tissue repair and angiogenesis by interacting with signaling molecules such as integrins and matrix metalloproteinases (MMPs). Dysregulated COL4A3 expression contributes to fibrosis and tumor metastasis by influencing extracellular matrix (ECM) remodeling. Knockout models show abnormal glomerular basement membranes, hematuria, and progressive renal failure, highlighting COL4A3's critical role in basement membrane assembly, tissue integrity, and kidney function.