Glomerular Basement Membrane (GBM; undissociated)

Cat. No.Available inproduct_image_23
159000.1 mg
159011 mg
 

Download the Antigen Specification in PDF format
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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 can allocate two baculovirus-derived forms of recombinant GBM antigen (undissociated, dissociated), whatever may be optimal for the respective assay approach (follow this link for the page with the details of the dissociated GBM antigen).