Glomerular Basement Membrane (GBM; dissociated)/肾小球基膜

Glomerular Basement Membrane (GBM; dissociated)

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.

相关疾病：肺出血肾炎综合症肺出血肾炎综合征是一种稀有并且严重的自身免疫疾病。能导致严重的肾和肺损伤并伴有急性肾小球性肾炎和肺出血，检测肾小球基底膜的自身抗体（anti-GBM）是一个可靠诊断的重要部分。主要的表位是IV胶原的α3链的球形NC1结构域。 肾小球基底膜（GBM）抗体能导致肾小球性肾炎或者肺出血。这两种疾病的结合就是肺出血肾炎综合症。抗GBM肾炎最为人所知的自身抗原就是所谓的肺出血肾炎抗原。1984年，这个抗原的生化特征为29kDa，肾小球基底膜的胶原酶抵抗分子。重要的表位处于IV型胶原酶的C端，后来发现是位于α3链的NC1结构域。肺出血肾炎表位是一个隐位，也就是说抗体优先结合一个变性的结构。这意味着在它天然六聚体的NC1结构域反应性很弱，一旦六聚体分解成单体或者二聚体后，表位才被暴露出来。

DIARECT为了适应实验的需要分别生产了两种从杆状病毒得到的重组GBM抗原（未分解的和分解的）。