GBM

Anti-GBM (short forglomerular basal membrane) auto-antibodies are markers for the diagnosis of anti-GBM disease. The target antigen of the autoantibodies, non-collagenous domain 1 of the α3-chain of type IV collagen (α3(IV)NC1) is localized in the glomerular basement membrane of the kidney and pulmonary alveoli. Together with podocytes and endothelial cells of the glomerulus, it constitutes the blood-urine barrier.

Anti-GBM disease is a type of small vessel vasculitis. The majority of patients suffers from rapidly progressive glomerulonephritis, as well as usually mild proteinuria and anuria. When both the kidneys and lungs are affected (as in 60 to 80 % of patients), the condition is known as Goodpasture's syndrome. If left untreated, anti-GBM disease can quickly lead to renal and lung failure, ultimately resulting in death. The diagnosis of anti-GBM disease in patients is based on the detection of anti-GBM autoantibodies. Up to 35% of patients with anti-GBM disease also have antibodies against the cytoplasm of neutrophil granulocytes (ANCA, mostly against myeloperoxidase (MPO)).

Virion\Serion’s GBM antigen (BA504R01), is recombinantly expressed in insect cells and purified via Strep-tag affinity chromatography, which guarantees high quality and availability. 

Please let us know if you are interested in 0.25 mg test sample.