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Goodpastures Syndrome

Goodpasture’s syndrome is a disease of unknown origin characterised by a pulmonary renal syndrome which include haemorrhage, glomerulonephritis and the presence of circulating antibodies against basement membrane antigens. Renal involvement can rapidly progress causing failure within a short timeframe from onset of disease. This can result in permanent renal failure.

A major autoantigen associated with Goodpasture’s is the non-collagenous domain 1 of collagen type IV. Antigens are generally purified from the kidney and work well as long as they are produced in eukaryotic systems. Anti-GBM antibodies have a prognostic value depending on their titre which can directly reflect severity. Antibodies to one of the two identified major epitopes causes toxicity and progression of the disease. Many patients with anti-GBM antibodies also have ANCA with specificity for MPO being the most common. 

References:
Lerner RA, Glassock RJ and Dixon FJ, J Exp Med 1967 126:989-1004
Sinico et al, Nephrol Dial Transplant 2006 21:397-401
Levy et al, Ann Intern Med 2001 134:1033-42
Segelmark M, Hellmark T and Wieslander J, Nephron Clin Pract 2003 94(3):59-68
Borza et al, J Biol chem 2005 280:27147-54
Hudson et al, New Engl J Med 2003 348:2543
 
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GBM

ATG02
Native - Bovine Kidney
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