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產品分類 > 自身免疫性風濕疾病ANA/ENA > SRP54/信號識別顆粒的54kDa亞基

SRP54/信號識別顆粒的54kDa亞基

產品貨號:18400/18401
促銷: 0.00

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0.1mg 1mg
德國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.

 

 

 

相關疾?。憾喟l性肌炎/皮肌炎 炎癥性的肌病以骨骼肌炎癥浸潤為特征。這些原發性的肌病包含一系列的共有的和非共有的并發癥。共同的亞類包含成人多發性肌炎(PM)和皮肌炎(DM),連同包含體肌炎,兒童肌病,惡性腫瘤相關的肌病和肌病重疊其他的自身免疫結締組織病。在30%的肌炎病人血清中發現了氨?;?tRNA合成酶的自身抗體。這些抗體紊亂有很高的特異性,并且與并發肺病高度相關??筍RP抗體與壞死性心肌炎綜合癥有關,這種疾病很難治愈。Mi-2自身抗體是皮肌炎的特異性血清學標志物。在大約20%的肌炎患者血清中檢測到,所以它可作為皮肌炎急性發作,預后良好和良好的治療反應的標記物。SRP54是信號識別顆粒(SRP)的一個54kDa的亞基,一種指導新合成的分泌蛋白從多聚核糖體轉運到內質網的胞質核糖核蛋白復合物。SRP由6種多肽和一種tRNA樣分子(7SL RNA)組成。SRP54亞基是一種GTP結合蛋白,并且直接結合初期的分泌器官和膜蛋白的信號序列??筍RP抗體存在于多發性肌炎患者體內,這種疾病是一種自身免疫性慢性肌肉炎癥。這些自身抗體主要識別SRP54亞基,并且能免疫沉淀很多SRP亞基和RNA組分。5%的皮肌炎患者抗SRP抗體為陽性,在Jo-1自身抗體陰性的患者中陽性率為18%。典型的抗SPR綜合癥是一種嚴重的多肌炎,發病為急性,且具攻擊性,伴隨肌痛,累及心臟,難以治愈,25%的患者只有5年的生存期。

DIARECT生產的全長重組人SRP54是在桿狀病毒表達系統中表達的。

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