中文名稱    多發性肌炎/硬皮病自身抗原2抗體    

英文名稱    EXOSC10

別    名    Autoantigen PM/Scl 2; Exosc10; Exosome component 10; EXOSX_HUMAN; P100 polymyositis scleroderma overlap syndrome associated autoantigen; P100 polymyositis-scleroderma overlap syndrome-associated autoantigen; p2; p3; p4; PM Scl; PM/Scl 100; PM/Scl-100; PMSCL; PMSCL2; Polymyositis/scleroderma autoantigen 100 kDa; Polymyositis/scleroderma autoantigen 2 100 kDa; Polymyositis/scleroderma autoantigen 2; RRP6; Rrp6p.      

供應商：遠慕生物

研究領域    細胞生物  表觀遺傳學      

抗體來源    Rabbit    

克隆類型    Polyclonal    

交叉反應    Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep,     

產品應用    WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    101kDa    

細胞定位    細胞核 細胞漿     

性    狀    Lyophilized or Liquid    

濃    度    1mg/ml    

免 疫 原    KLH conjugated synthetic peptide derived from human EXOSC10/PMSCL2:41-140/885     

亞    型    IgG    

純化方法    affinity purified by Protein A    

儲 存 液    0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.    

保存條件    Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.    

PubMed    PubMed    

產品介紹    The exosome is a multi-subunit complex composed of several highly conserved proteins, some of which are 3’ to 5’ exoribonucleases. The complex is involved in a variety of cellular processes and is responsible for degrading unstable mRNAs that contain AU-rich (ARE) elements in their untranslated 3’ region. EXOSC10, also known as PMSCL, PMSCL2, p2, p3, p4, RRP6, Rrp6p, PM-Scl, or PM/Scl-100, is an 885 amino acid protein that contains one HRDC domain and one 3’-5’ enonuclease domain. Localized to both the cytoplasm and the nucleus, EXOSC10 is part of the post-splicing exosome complex and is involved in mRNA surveillance, mRNA nuclear export and nonsense-mediated decay of mRNAs containing premature stop codons. Antibodies against EXOSC10 have been found in patients with scleroderma and/or polymyositis (chronic diseases of the skin and muscle, respectively), suggesting that EXOSC10 may be involved in the pathogenesis of these diseases. Two isoforms of EXOSC10 exist due to alternative splicing events.    

抗原與抗體的關系

①抗體和相應的抗原結合后，可以促進白細胞的吞噬作用而將抗原消除，使抗原失去致病作用。

②一種抗體只能抵抗一種抗原，而且一種抗體只能受相應的抗原刺激后才能形成。

免疫的功能：

免疫具有三方面的功能：

①防御功能：抵抗抗原的侵入、防止疾病的產生。

②自我穩定：清除體內衰老、死亡和損傷的細胞。

③免疫監視：監視、識別和清除體內產生的異常細胞。

特別提醒：過敏反應：當人體抵抗抗原侵入的功能過強時，在過敏原(引起過敏反應的物質，如某些食物、藥物)的刺激下，人體就會發生過敏反應。

1.抗體名稱容易出錯

抗體一般以抗原來命名，名稱核對時最簡單也最容易犯錯，有些蛋白質有不同亞基（HIF-1α、HIF-1β）；有些蛋白質有不同磷酸化狀態，例如EGFR和p-EGFR的區別，等等。

除了蛋白質的名稱外，有其他后綴。

2.多抗和單抗不同

單抗上面介紹過了，而多抗(polyclonal antibodies)一般用兔子、山羊等大型動物，因為多抗是直接從血液中純化提取出來的，而不像單抗是用細胞培養出來的。

單克隆抗體的優點是產量持續穩定、特異性較高、但靈敏度不如多抗。如果對抗體的特異性要求高，用量較大或需要長期使用一致的抗體，制備的抗體應用要求多（WB/IP/IF/ICC等)，可以選擇單克隆抗體。

多抗的特異性較差，存在批間差(因為兔子的血被放光掛掉了，而山羊可以常年養著，放點血出來，在養幾天再放血，好殘忍)，易造成背景，例如在WB中有雜帶，在IHC中背景較深等等。但由于多抗識別多個抗原表位，對于豐度偏低的蛋白也更容易檢出。