Glomerular Basement Membrane (GBM; dissociated)
| Cat. No. | Available in | ![]() |
| 16800 | 0.1 mg | |
| 16801 | 1.0 mg | |
Diseases:
Goodpasture Syndrome
Antibodies to GBM can cause glomerulonephritis or pulmonary hemorrhage; the combination of these 2 clinical symptoms is known as Goodpasture syndrome. The best known autoantigen in anti-GBM nephritis is the so-called Goodpasture antigen.
Two decades ago in 1984, the antigen was biochemically characterized and shown to be a 29 kDa collagenase-resistant molecule of the glomerular basement membrane. The important epitope is at the C-terminal end of type IV collagen and was later shown to be localized to the NC1 domain of the α3(IV) chain.
The Goodpasture epitope is a so-called cryptotope, i.e. the antibodies preferentially bind to a denatured structure. That means the NC1 domain in its native hexameric form is of poor reactivity; once the hexamer is dissociated into monomers and dimers, the epitope is exposed. DIARECT makes allowance for this fact and offers a recombinant, dissociated GBM antigen, which is produced in the baculovirus expression system.


