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PM/Scl100

PM/Scl100

产品货号:16000/16001
促销: 0.00
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0.1mg 1mg
德国Diarect

PM/Scl100
Diseases:
Scleroderma Polymyositis and Dermatomyositis 

PM/Scl 100 is a nucleolar protein and represents one crucial component of the so-called "PM/Scl complex" or "nuclear exosome", which is an RNA-processing complex consisting of a number of exoribonucleases (not to be confused with certain multivesicular bodies which are shed by cultured cells into the culture fluid and are also called exosomes).

Autoantibodies against PM/Scl 100 occur in patients suffering from polymyositis, scleroderma or polymyositis/scleroderma overlap syndromes. While antibodies to other components of the exosome complex (PM/Scl 75 is available from DIARECT by special order) can be found in some sera, practically all patients with autoantibodies against the PM/Scl complex are positive for PM/Scl 100 antibodies, making this specificity an important marker. The presence of PM/Scl 100 autoantibodies is a comparatively "good" prognostic sign in polymyositis/scleroderma overlap syndrome, unlike the poor prognosis seen when other myositis- and systemic sclerosis-specific autoantibodies are present.

PM/Scl 100 from DIARECT is expressed in the baculovirus expression system.

 

 相关疾病:多发性肌炎,硬皮病

多发性肌炎/皮肌炎

炎症性的肌病以骨骼肌炎症浸润为特征。这些原发性的肌病包含一系列的共有的和非共有的并发症。共同的亚类包含成人多发性肌炎(PM)和皮肌炎(DM),连同包含体肌炎,儿童肌病,恶性肿瘤相关的肌病和肌病重叠其他的自身免疫结缔组织病。在30%的肌炎病人血清中发现了氨酰基-tRNA合成酶的自身抗体。这些抗体紊乱有很高的特异性,并且与并发肺病高度相关。抗SRP抗体与坏死性心肌炎综合症有关,这种疾病很难治愈。Mi-2自身抗体是皮肌炎的特异性血清学标志物。在大约20%的肌炎患者血清中检测到,所以它可作为皮肌炎急性发作,预后良好和良好的治疗反应的标记物。系统性硬化病/肢端硬皮综合症系统性硬化病(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。 PM/Scl 100是一种核蛋白,它是PM/Scl复合物或叫核内外核体(RNA加工复合体)的一种重要的组分,PM/Scl复合物是RNA加工复合物,包含很多外切核糖核酸酶(不要与某些会从培养的细胞中流出到培养液中的多泡体,也叫外核体的混淆)。抗PM/Scl 100抗体出现在多发性肌炎患者,硬皮病患者或者多发性肌炎和硬皮病重叠综合症患者的体内。外核体中其他组分的抗体(PM/Scl 75,DIARECT特殊订购)在一些血清中也可以发现,尤其是所有含抗PM/Scl复合物抗体,且PM/Scl 100抗体也阳性的患者血清中,所以它可以作为一种特异性的重要标志物。对多发性肌炎和硬皮病重叠综合症患者来说,检测到抗PM/Scl 100抗体相对来说是一个好的预后诊断信号,不像检测到其他肌炎或系统性硬化症特异性的自身抗体时代表预后不良。DIARECT生产的PM/Scl 100抗原是在杆状病毒系统中表达的。