Sp100

Diseases:

Primary Biliary Cirrhosis

Primary biliary cirrhosis (PBC) is an autoimmune chronic cholestatic liver disease characterized by destruction of intrahepatic bile ducts and development of cirrhosis and liver failure. Serologically, PBC is characterized by the presence of antimitochondrial (AMA, M2) antibodies. The immunological profil of PBC also includes antinuclear antibodies (ANA), presenting two distinct patterns, multiple nuclear dots (MND) and rim-like/membranous patterns in IIF. Sp100 and promyelocytic leukemia antigen account for the MND pattern, and gp210, nucleoporin p62 and lamin B receptor are associated with nuclear membrane staining.

Sp100 is the main antigenic target of MND. This 54-kDa nuclear protein has transcription-enhancing activity and is upregulated by interferon. Although the high specificity of anti-gp210 for PBC is generally accepted, the true diagnostic role of Sp100 is still under debate since also a number of SLE sera and those of patients with other hepatic or non-hepatic disorders seem to be seropositive for Sp100 antibodies. In any case, anti-Sp100 is more frequent in liver patients and has been significantly more often detected in PBC, for which it can be regarded as a highly specific serological marker.

Sp100 antigen from DIARECT is produced in the baculovirus / insect cell expression system.

相关疾病：原发性胆汁性肝硬变原发性胆汁肝硬化（PBS）是一种慢性的自身免疫胆汁淤积肝疾病，以损坏肝内胆管为特征，并导致肝硬化和肝衰竭。临床特征包括疲劳、搔痒和黄疸。PBC的血清学特征是抗线粒体（AMA，M2）抗体。PBC的免疫特征还包括抗核抗体（ANA），在IIF检测中显示出两个不同的类型，多核点型（MND）和核膜型。Sp100和前髓细胞性白血病抗原为MND型，gp210，核孔p62和核纤层蛋白B受体与核膜型相关。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。Sp100是多核点型（MND）的主要靶抗原。这种54kDa的核蛋白有转录增强活性，在干扰素作用下活性上调。虽然抗gp210抗体在原发性胆汁肝硬化病中有很高的特异性，但是Sp100在诊断中的作用还在争论中，因为很多SLE和其他肝病或非肝紊乱病患者的血清也显示Sp100阳性。不管怎样，抗Sp100抗体在肝病患者血清中更常见，并在原发性胆汁肝硬化患者血清中更经常被检测到，可以作为一种高特异性的血清学标志。

 DIARECT生产的Sp100抗原在杆状病毒/昆虫细胞表达系统中表达。