多发性肌炎/硬皮病自身抗原2抗体说明书

?中文名称 多发性肌炎/硬皮病自身抗原2抗体 英文名称 EXOSC10 别 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. 供应商：远慕生物 研究领域 细胞生物 表观遗传学 抗体来源 Rabbit 克隆类型 Polyclonal 交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, 产品应用 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 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. 分 子 量 101kDa 细胞定位 细胞核 细胞浆 性 状 Lyophilized or Liquid 浓 度 1mg/ml 免 疫 原 KLH conjugated synthetic peptide derived from human EXOSC10/PMSCL2:41-140/885 亚 型 IgG 纯化方法 affinity purified by Protein A 储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. 保存条件 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 产品介绍 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. 抗原与抗体的关系 ①抗体和相应的抗原结合后，可以促进白细胞的吞噬作用而将抗原消除，使抗原失去致病作用。 ②一种抗体只能抵抗一种抗原，而且一种抗体只能受相应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、防止疾病的产生。 ②自我稳定：清除体内衰老、死亡和损伤的细胞。 ③免疫监视：监视、识别和清除体内产生的异常细胞。 特别提醒：过敏反应：当人体抵抗抗原侵入的功能过强时，在过敏原(引起过敏反应的物质，如某些食物、药物)的刺激下，人体就会发生过敏反应。 1.抗体名称容易出错 抗体一般以抗原来命名，名称核对时最简单也最容易犯错，有些蛋白质有不同亚基（HIF-1α、HIF-1β）；有些蛋白质有不同磷酸化状态，例如EGFR和p-EGFR的区别，等等。 除了蛋白质的名称外，有其他后缀。 2.多抗和单抗不同 单抗上面介绍过了，而多抗(polyclonal antibodies)一般用兔子、山羊等大型动物，因为多抗是直接从血液中纯化提取出来的，而不像单抗是用细胞培养出来的。 单克隆抗体的优点是产量持续稳定、特异性较高、但灵敏度不如多抗。如果对抗体的特异性要求高，用量较大或需要长期使用一致的抗体，制备的抗体应用要求多（WB/IP/IF/ICC等)，可以选择单克隆抗体。 多抗的特异性较差，存在批间差(因为兔子的血被放光挂掉了，而山羊可以常年养着，放点血出来，在养几天再放血，好残忍)，易造成背景，例如在WB中有杂带，在IHC中背景较深等等。但由于多抗识别多个抗原表位，对于丰度偏低的蛋白也更容易检出。