FITC标记的多发性肌炎/硬皮病自身抗原2抗体
产品名称： FITC标记的多发性肌炎/硬皮病自身抗原2抗体
英文名称： Anti-EXOSC10/FITC
产品编号： HZ-13120R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-EXOSC10/FITC Conjugated antibody
FITC标记的多发性肌炎/硬皮病自身抗原2抗体
| 英文名称 | Anti-EXOSC10/FITC |
| 中文名称 | FITC标记的多发性肌炎/硬皮病自身抗原2抗体 |
| 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. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 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 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 101kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human EXOSC10/PMSCL2 |
| 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. |
| 产品介绍 | background: 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. Function: Putative catalytic component of the RNA exosome complex which has 3'->5' exoribonuclease activity and participates in a multitude of cellular RNA processing and degradation events. In the nucleus, the RNA exosome complex is involved in proper maturation of stable RNA species such as rRNA, snRNA and snoRNA, in the elimination of RNA processing by-products and non-coding 'pervasive' transcripts, such as anti-sense RNA species and promoter-upstream transcripts (PROMPTs), and of mRNAs with processing defects, thereby limiting or excluding their export to the cytoplasm. The RNA exosome may be involved in Ig class switch recombination (CSR) and/or Ig variable region somatic hypermutation (SHM) by targeting AICDA deamination activity to transcribed dsDNA substrates. In the cytoplasm, the RNA exosome complex is involved in general mRNA turnover and specifically degrades inherently unstable mRNAs containing AU-rich elements (AREs) within their 3' untranslated regions, and in RNA surveillance pathways, preventing translation of aberrant mRNAs. It seems to be involved in degradation of histone mRNA. EXOSC10 has 3'-5' exonuclease activity (By similarity). EXOSC10 is required for nucleolar localization of C1D and probably mediates the association of SKIV2L2, C1D and MPP6 wth the RNA exosome involved in the maturation of 5.8S rRNA. Subunit: Component of the RNA exosome complex. The catalyticallyinactive RNA exosome core (Exo-9) complex is believed to associatewith catalytic subunits EXOSC10, and DIS3 or DIS3L incytoplasmic-and nuclear-specific RNA exosome complex forms.Interacts with C1D and MPHOSPH6. Subcellular Location: Cytoplasm. Nucleus > nucleolus. Nucleus. Strongly enriched in the nucleolus and a small amount has been found in cytoplasm supporting the existence of a nucleolar RNA exosome complex form. Similarity: Contains 1 3'-5' exonuclease domain. Contains 1 HRDC domain. Database links: Entrez Gene: 5394 Human Entrez Gene: 50912 Mouse Omim: 605960 Human SwissProt: Q01780 Human SwissProt: P56960 Mouse Unigene: 632368 Human Unigene: 266635 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
外切体是由一些高度保守的蛋白质组成的多亚基复合物，其中一些是3’至5’外切核糖核酸酶。该复合物参与多种细胞过程，并负责降解不稳定的mRNA，其中含有丰富的AU（ARE）元素在其未翻译的3'区。EXOSC10又称PMSCL、PMSCL2、p2、p3、p4、RRP6、Rrp6p、PM-Scl或PM/Scl-100，是一种885个氨基酸的蛋白质，包含一个HRDC结构域和一个3'-5'烯醇化酶结构域。EXOSC10定位于细胞质和核，是剪接后外体复合物的一部分，参与mRNA监测、mRNA核输出和含有过早终止密码子的mRNA的无义衰变。在硬皮病和/或多发性肌炎（分别是皮肤和肌肉的慢性疾病）患者中发现了抗EXOSC10的抗体，表明EXOSC10可能参与了这些疾病的发病机制。EXOSC10的两种异构体由于选择性剪接事件而存在。