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EXOSC10多发性肌炎/硬皮病自身抗原2抗体

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  • EXOSC10多发性肌炎/硬皮病自身抗原2抗体
    英文名称 EXOSC10
    中文名称 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/1380元 购买    200ul/2200元 购买    大包装/询价
    说 明 书 100ul  200ul
    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 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.

    SWISS:
    Q01780

    Gene ID:
    5394 

    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. 
     
    产品图片
    Tissue/cell: human skin tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; 
    Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; 
    Incubation: Anti-EXOSC10, Unconjugated(bs-13120R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining


    Tissue/cell: Mouse Lymph nodes; 4% Paraformaldehyde-fixed and paraffin-embedded; 
    Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; 
    Incubation: Anti-EXOSC10, Unconjugated(bs-13120R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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