多發(fā)性肌炎/硬皮病自身抗原2抗體說(shuō)明書

中文名稱    多發(fā)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(yīng)商：遠(yuǎn)慕生物

研究領(lǐng)域    細(xì)胞生物  表觀遺傳學(xué)      

抗體來(lái)源    Rabbit    

克隆類型    Polyclonal    

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

產(chǎn)品應(yīng)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(fù)） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    101kDa    

細(xì)胞定位    細(xì)胞核 細(xì)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(chǔ) 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(chǎn)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(guān)系

①抗體和相應(yīng)的抗原結(jié)合后，可以促進(jìn)白細(xì)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(yīng)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(chǎn)生。

②自我穩(wěn)定：清除體內(nèi)衰老、死亡和損傷的細(xì)胞。

③免疫監(jiān)視：監(jiān)視、識(shí)別和清除體內(nèi)產(chǎn)生的異常細(xì)胞。

特別提醒：過(guò)敏反應(yīng)：當(dāng)人體抵抗抗原侵入的功能過(guò)強(qiáng)時(shí)，在過(guò)敏原(引起過(guò)敏反應(yīng)的物質(zhì)，如某些食物、藥物)的刺激下，人體就會(huì)發(fā)生過(guò)敏反應(yīng)。

1.抗體名稱容易出錯(cuò)

抗體一般以抗原來(lái)命名，名稱核對(duì)時(shí)最簡(jiǎn)單也最容易犯錯(cuò)，有些蛋白質(zhì)有不同亞基（HIF-1α、HIF-1β）；有些蛋白質(zhì)有不同磷酸化狀態(tài)，例如EGFR和p-EGFR的區(qū)別，等等。

除了蛋白質(zhì)的名稱外，有其他后綴。

2.多抗和單抗不同

單抗上面介紹過(guò)了，而多抗(polyclonal antibodies)一般用兔子、山羊等大型動(dòng)物，因?yàn)槎嗫故侵苯訌难褐屑兓崛〕鰜?lái)的，而不像單抗是用細(xì)胞培養(yǎng)出來(lái)的。

單克隆抗體的優(yōu)點(diǎn)是產(chǎn)量持續(xù)穩(wěn)定、特異性較高、但靈敏度不如多抗。如果對(duì)抗體的特異性要求高，用量較大或需要長(zhǎng)期使用一致的抗體，制備的抗體應(yīng)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?yàn)橥米拥难环殴鈷斓袅耍窖蚩梢猿Ｄ牮B(yǎng)著，放點(diǎn)血出來(lái)，在養(yǎng)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(shí)別多個(gè)抗原表位，對(duì)于豐度偏低的蛋白也更容易檢出。