The anti-GBM antibodies are targeted to the globular C terminal non-collagenous domain 1 (NC1) of the α3 chain of collagen IV. They bind along the Glomerular Basement Membrane as a characteristic continuous layer which is demonstrable by Immunofluorescence on kidney sections.
The anti-GBM antibodies are associated to the Goodpasture Syndrome which is characterized by glomerulonephritis and pulmonary bleeding. It is a medical emergency with a high fatality rate if not treated. The anti-GBM antibodies are the primary pathogenic autoantibodies, inducing Glomerulonephritis, in all patients with Goodpasture Syndrome. Their detection allows differentiation of the Goodpasture Syndrome from other causes of Glomerular Nephritis and Pulmonary Bleeding.
Recombinant, human, expressed in Baculovirus infected Sf9 cells (for more information on our antigens click here)