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GBM -Ab EIA Auto-Immune Markers 013-GBM-48 Glomerular Basement Membrane

ProductsGBM -Ab EIA Auto-Immune Markers 013-GBM-48 Glomerular Basement Membrane
Company ALPCO Diagnostics
Item GBM -Ab EIA Auto-Immune Markers 013-GBM-48 Glomerular Basement Membrane
Features 
Description This Immunoscan Anti-GBM was developed to detect the presence of antibodies against the glomerular basement membrane. The information is clinically useful in diagnosing Goodpastures syndrome, where anti-collagen (IV) - a3 chain antibody formation along with glomerulonephritis and pulmonary hemorrhage are primary diagnostic parameters. Since there are several other conditions presenting with similar symptoms, developed independently from anti-glomerular basement membrane (GBM) antibodies, this test can help to differentiate between these autoimmune renal disorders. The antigen used in the test is the M2 subunit purified from the noncollagenous (NC1) domain of type IV collagen. This antigen is fully described in the review article Biology of Disease Goodpasture Syndrome: Molecular Architecture and Function of Basement Membrane Antigen (1). Recent studies have shown that all Goodpasture patients have antibodies directed against M2 and some patients have additional antibodies against other basement membrane constituents and/or other collagen IV domains. The use of this purified antigen does not give false positive results for patient specimens containing other autoantibodies to the GBM, in contrast to crude collagenase digests of the total GBM (2). Glomerulonephritis is a major cause of irreversible renal failure. Many forms of glomerulonephritis are caused by immunological mechanisms, whereby a person forms antibodies against his own tissue components (auto-immunity) (3). Dixon and coworkers (4, 5) showed that in one particular type of glomerulonephritis, originally described by Ernest Goodpasture in 1919 (6) and subsequently referred to as Goodpastures syndrome (7), the disease was caused by an antibody response against antigens present in the glomerular basement membrane (GBM). Thus the disease is sometimes referred to as anti-GBM mediated glomerulonephritis. While Goodpastures syndrome is a relatively rare condition, the disease is rapidly progressive and, unless treated, has a case-fatality rate of 75-90% (8). Immediate and correct treatment is necessary before kidney (and often lung) damage has progressed too far. In clinical practice the diagnosis and recognition of the disease is frequently delayed since it is unexpected. Clinical signs such as hemoptysis, precipitate anemia, pulmonary opacities at x-ray and/or oliguria are in a sense unspecific and common to a variety of disorders. Mild prodromal lung or kidney symptoms are often overlooked or masked by a precipitating infection. The Immunoscan Anti-GBM offered is based on ELISA methodology (15, 16). The test utilizes wells coated with purified GBM antigen. Diluted patient serum is applied to the well and incubated. If specific anti-GBM antibodies are present they will bind to the antigen. Unbound material is washed away and any bound antibody is detected by adding enzyme-labeled anti-human IgG, followed by a second washing step and incubation with substrate. A well containing anti-GBM antibody will develop a color, which can be quantified.
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