What are Anti GBM antibodies?
Anti GBM antibodies are targeted toward the noncollagenous domain 1 (NC1) domain of the α3 chain of type IV collagen, which is a component of the GBM.
Formation of the type IV collagen network in normal GBM sequesters these epitopes from the immune system, preventing tolerance during fetal development.
Anti GBM associated disease occurs after the kidney is injured in a manner that exposes these regions of the collagen molecule; the collagen is not recognized as “self” and generates an immune response.
Anti GBM antibodies are found in 90% to 95% of patients with Goodpasture disease and are deposited in the GBM.
On kidney biopsy immunofluorescence, they form linear deposits of immunoglobulin along the basement membranes.