gp210

Diseases:  
Primary Biliary Cirrhosis 

The nuclear envelope separates cytoplasm from the nuclear compartment in eukaryotic cells. It is composed of a double membrane, pore complexes, and the nuclear lamina. Among the best characterized pore complex proteins are two distinct glycoproteins with molecular masses of 62 and 210 kDa. The latter, termed gp210, is an integral membrane protein that carries high mannose-type oligosaccharides in its large luminal section. gp210 is believed to be involved in the formation of nuclear pores and the attachment of pore complex constituents to the nuclear membrane.

Although M2 autoantibodies can be qualified as the most characteristic serological marker for PBC, there are a number of antibodies against nuclear components present in sera of up to 50% of these patients. A perinuclear staining pattern by indirect immunoflurescence is indicative of the reactivity of the sera with the components of the nuclear envelope. Antibodies directed against gp210 are found in about 25 % of patients with AMA/M2-positive PBC and up to 50% of those with M2-negative PBC. The disease specificity of anti-gp210 is close to 100%. In contrast to M2 antibodies, gp210 antibodies have prognostic significance and are thought to correlate with progression toward end-stage liver disease. The relevant epitopes are located in the short cytoplasmic portion of the gp210 protein.

DIARECTs recombinant gp210 antigen has been optimally designed for assay performance and high-level expression of the diagnostically relevant epitopes in the baculovirus/insect cell system.

相关疾病：原发性胆汁性肝硬变原发性胆汁肝硬化（PBS）是一种慢性的炎症性自身免疫肝病，病理表现包括肝内胆管的阻塞，干扰胆汁的分泌，导致纤维化和肝硬化。临床特征包括疲劳、搔痒和黄疸。在90-95% 的PBC病人血清中可检测到抗线粒体的抗体（M2），它们和其他的一些核内以及核膜的抗原（Sp100，gp210），都是重要的标记物。除了特异性自身抗体，其他的两个主要检测方面包括相容性的肝脏组织学和胆汁阻塞模型肝功能测试也用于PBC诊断。核被膜将细胞质从真核细胞的核室中分离出来。它由一个双层膜，核孔复合体和核纤层组成 。在这些具特征的核孔复合体蛋白中， 有两个不同的蛋白，分子量分别为62和210kDa。后者称作gp210，是一个膜内在蛋白质，在它的大网眼截面携带富含甘露糖的低聚糖。gp210被认为参与核孔的形成和使核孔复合体组分粘到核膜上。    虽然M2自身抗体可以作为PBC具特征性的血清学标志，但很多抗核成分的抗体在高达50%的这些病人血清中都存在。核周染色的间接免疫荧光检测指示了有核被膜组分的血清反应性。抗gp210抗体存在于25%的AMA/M2阳性的原发性胆汁肝硬化患者血清中，在M2阴性的原发性胆汁肝硬化患者血清中高达到50%。抗gp210抗体对该疾病的特异性接近100%。与M2抗体相比，gp210抗体有预后意义，被认为与末期肝病进展有关。相关表位位于gp210蛋白的短细胞质部分。

DIARECT生产的重组gp210抗原经过优化设计适于实验分析并在杆状病毒/昆虫细胞表达系统中高水平表达特征性相关表位。