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产品分类 > 自免疾病检测原料 > DNA Topoisomerase I (Scl-70; full length)/DNA拓扑异构酶Ⅰ(Scl-70;全长)

DNA Topoisomerase I (Scl-70; full length)/DNA拓扑异构酶Ⅰ(Scl-70;全长)

产品货号:12400/12401
促销: 0.00

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0.1mg 1mg
德国Diarect

DNA Topoisomerase I (Scl-70; full length)


Diseases:
Scleroderma
 

DNA toposisomerase I is a key nuclear enzyme that interconverts supercoiled DNA to the required topological conformations for normal DNA replication and transcription. This enzyme is the target antigen for the so-called Scl-70 autoantibodies. Scl-70 antibodies are a specific marker in scleroderma patients (specificity 98-100%) and are associated with the presence of diffuse skin involvement and pulmonary fibrosis.

In human tissues the DNA topoisomerase I is initially synthesized as a protein with a molecular weight of 100 kDa. Most of this precursor is then proteolytically processed to a 70-kDa size, from which the Scl-70 antigen has derived its name.

DNA topoisomerase I (Scl-70) antigens from DIARECT are produced in the baculovirus / insect cell expression system. To allow optimal assay development DIARECT offers two distinct DNA topoisomerase I antigens: one is the full-length 100-kDa enzyme precursor , the other one is a truncated form resembling the processed 70-kDa form of the antigen.

 

相关疾病:硬皮病/CREST综合征系统性硬化病(SSc)是一种结缔组织全身紊乱疾病,影响皮肤和内部器官,以外周动脉粥样硬化和内脏结构纤维化为特征。在皮肤和内脏的细胞外基质(主要是胶原)不同程度的积累,系统性硬化病常常和特异性自身抗体相关,尤其是着丝粒抗体和Scl-70抗体(DNA拓扑异构酶I的自身抗体)。其他类型的自身抗体,如抗PM/Scl抗体,认为是伴有肌炎、硬皮病和PM/Scl重叠综合症的另一亚类自身免疫病人的特征。硬皮病可能导致心、肺、肾和肠胃道的纤维化和功能衰退。DNA拓扑异构酶I是一种关键的核酶,使超螺旋的DNA转变为正常DNA复制和转录需要的拓扑结构。这个酶是叫做Scl-70自身抗体的靶抗原,Scl-70抗体是硬皮病患者的特异性标志(特异性98-100%),并且与弥漫性皮肤病和肺纤维化有关。在人类组织中,DNA拓扑异构酶I最初合成的是分子量100kDa的蛋白,大多数合成过程后面出现蛋白质水解,形成分子量为70kDa的蛋白,Scl-70抗原的名字由此而来。

DIARECT生产的DNA拓扑异构酶I(Scl-70)抗原是在杆状病毒/昆虫系统中表达的。为了实现最理想的实验效果,DIARECT提供了两种不同的DNA拓扑异构酶I(Scl-70)抗原,一种是全长的,分子量为100kDa的酶前体,另一种是截短的,经过处理合成的分子量为70kDa抗原。