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Mi-2

產品貨號:18100/18101
促銷: 0.00

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0.1mg 1mg
德國Diarect

Mi-2

Diseases:
Polymyositis and Dermatomyositis

The Mi-2? autoantigen (MW 221.3 kDa) belongs to a family of so-called chromodomain helicase-DNA-binding proteins; several isoforms of Mi-2 exist with molecular weights between 210 and 240 kDa. Biochemically, Mi-2? is a component of the nucleosome remodeling and deacetylase (NuRD) complex, which seems to be involved in transcription regulation.

Autoantibodies targeting the Mi-2 nuclear antigen are a serologic feature of idiopathic inflammatory myopathies (IIM). In IIM Mi-2 antibodies are characterized by diagnostic sensitivity and specificity of approximately 4-18% and 98-100%, respectively. Furthermore, anti-Mi-2 antibodies are strongly associated with dermatomyositis (frequency up to 31%) and have a very high positive predictive value for such a disease subset. Anti-Mi-2 are the only defined myositis-specific autoantibodies clearly directed to a nuclear target. Another rather exceptional feature of Mi-2 antibodies relates to their frequency in children, which is comparable to that in adults.

Previously, only several recombinant Mi-2? subfragments expressed in E.coli have been used in diagnostic applications. Recombinant, human Mi-2? from DIARECT is a full-length, 220-kDa autoantigen, eukaryotically expressed in baculovirus-infected insect cells. DIARECT is the first commercial supplier offering a recombinant full-length Mi-2 antigen. 

 

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。Mi-2?自身抗原(分子量 221.3 kDa)屬于染色質域解旋酶DNA結合蛋白家族,Mi-2有幾個亞型,分子量在210到240kDa之間。Mi-2?是核小體重塑和脫乙?;福∟uRD)復合物的一個組分,可能參與轉錄調節。靶向Mi-2核抗原的自身抗體是自發性肌炎(IIM)存在的一個血清學特性。IIM Mi-2抗體以診斷靈敏性和各自約4-18%和98-100%的特異性為特征。此外,抗Mi-2的抗體與皮肌炎強烈相關(頻率高達31%),對于這種疾病亞型有非常高的陽性預測價值??筂i-2抗體是唯一確認的直接指向核靶點的肌炎特異性自身抗體。Mi-2抗體另外一個較為罕見特性就是在兒童中出現的頻率與在成人中出現的頻率相當。

以前,只有少數在E.coli表達的重組Mi-2 ?亞片段可以應用于診斷。DIARECT生產的重組人Mi-2 ?是220kD全長的抗原,在桿狀病毒感染的昆蟲細胞中真核表達。DIARECT是第一個提供商業重組全長Mi-2抗原的公司。

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