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产品分类 > 自免疾病检测原料 > SRP54/信号识别颗粒的54kDa亚基

SRP54/信号识别颗粒的54kDa亚基

产品货号:18400/18401
促销: 0.00

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0.1mg 1mg
德国Diarect

SRP54

Diseases:
Polymyositis and Dermatomyositis 

SRP54 is a 54-kDa subunit of the signal recognition particle (SRP), a cytoplasmic ribonucleoprotein complex that directs the translocation of newly synthesized secretory proteins from the polysome to the endoplasmic reticulum. SRP is composed of six polypeptides and a tRNA-like molecule known as 7SL RNA. The SRP54 subunit is a GTP-binding protein and directly binds the signal sequences of nascent secretory and membrane proteins.

 

Anti-SRP autoantibodies occur in patients with polymyositis, an autoimmune chronic muscle inflammation. These auto-antibodies predominantly recognize the SRP54 subunit and can also immunoprecipitate several of the SRP subunits and the RNA component. About 5% of myositis patients are positive for anti-SRP autoantibodies, rising to 18% in the subgroup of Jo-1 autoantibody-negative patients. The classic anti-SRP syndrome is a severe form of polymyositis in which the myositic inflammation is acute and aggressive in onset, with common myalgias and cardiac involvement. A poor response to therapy is normally observed, with a 5-year survival rate in the range of 25%.


DIARECT produces full-length recombinant human SRP54 in the baculovirus/Sf9 expression system.

 

 

 

相关疾病: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。SRP54是信号识别颗粒(SRP)的一个54kDa的亚基,一种指导新合成的分泌蛋白从多聚核糖体转运到内质网的胞质核糖核蛋白复合物。SRP由6种多肽和一种tRNA样分子(7SL RNA)组成。SRP54亚基是一种GTP结合蛋白,并且直接结合初期的分泌器官和膜蛋白的信号序列。抗SRP抗体存在于多发性肌炎患者体内,这种疾病是一种自身免疫性慢性肌肉炎症。这些自身抗体主要识别SRP54亚基,并且能免疫沉淀很多SRP亚基和RNA组分。5%的皮肌炎患者抗SRP抗体为阳性,在Jo-1自身抗体阴性的患者中阳性率为18%。典型的抗SPR综合症是一种严重的多肌炎,发病为急性,且具攻击性,伴随肌痛,累及心脏,难以治愈,25%的患者只有5年的生存期。

DIARECT生产的全长重组人SRP54是在杆状病毒表达系统中表达的。