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ERAL1 Recombinant Monoclonal Antibody

  • 中文名稱：
    ERAL1 Recombinant Monoclonal Antibody
  • 貨號(hào)：
    CSB-RA007759MA1HU
  • 規(guī)格：
    ￥1320
  • 圖片：
    • IHC image of CSB-RA007759MA1HU diluted at 1:200 and staining in paraffin-embedded human small intestine tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a Goat anti-human polymer IgG labeled by HRP and visualized using 0.05% DAB.
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品描述：
    ERAL1 Recombinant Monoclonal Antibody（CSB-RA007759MA1HU）是一款針對(duì)線粒體核糖體組裝因子ERAL1靶點(diǎn)開發(fā)的高特異性抗體。ERAL1作為線粒體核糖體小亞基的伴侶蛋白，在維持線粒體RNA穩(wěn)定性及核糖體組裝中發(fā)揮關(guān)鍵作用，其表達(dá)異常與能量代謝紊亂及細(xì)胞凋亡調(diào)控密切相關(guān)。本產(chǎn)品經(jīng)嚴(yán)格驗(yàn)證，可精準(zhǔn)識(shí)別內(nèi)源性ERAL1蛋白，適用于ELISA和免疫組化（IHC）等實(shí)驗(yàn)，其中IHC推薦使用稀釋比例為1:50-1:200，實(shí)驗(yàn)數(shù)據(jù)顯示其在多種組織樣本中呈現(xiàn)清晰的定位信號(hào)與低背景干擾。該抗體適用于探究線粒體功能調(diào)控機(jī)制、細(xì)胞代謝相關(guān)通路研究以及疾病模型中ERAL1表達(dá)水平變化的分析，為解析線粒體相關(guān)疾病的分子機(jī)制提供可靠工具。其穩(wěn)定性和重復(fù)性已通過多批次質(zhì)檢驗(yàn)證，適用于基礎(chǔ)科研領(lǐng)域中對(duì)線粒體核糖體組裝、RNA加工及能量代謝過程的分子生物學(xué)研究。
  • Uniprot No.：
  • 基因名：
    ERAL1
  • 別名：
    CEGA antibody; Conserved ERA like GTPase antibody; Conserved ERA-like GTPase antibody; ERA antibody; Era (E. coli G protein homolog) like 1 antibody; Era G protein like 1 (E. coli) antibody; Era G protein like 1 antibody; ERA W antibody; ERA-like protein 1 antibody; ERA-W antibody; Eral1 antibody; ERAL1_HUMAN antibody; ERAL1A antibody; ERAW antibody; GTP binding protein era homolog antibody; GTPase Era antibody; GTPase Era; mitochondrial antibody; GTPase human homolog of E. coli essential cell cycle protein Era antibody; H-ERA antibody; HERA A antibody; hERA antibody; HERA B antibody; HERAA antibody; HERAB antibody; mitochondrial antibody
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human ERAL1 protein
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Non-conjugated
  • 克隆類型：
    Monoclonal
  • 抗體亞型：
    hIgG1
  • 純化方式：
    Affinity-chromatography
  • 克隆號(hào)：
    27A9
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:50-1:200
  • Protocols：
  • 儲(chǔ)存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 功能：
    Probable GTPase that plays a role in the mitochondrial ribosomal small subunit assembly. Specifically binds the 12S mitochondrial rRNA (12S mt-rRNA) to a 33 nucleotide section delineating the 3' terminal stem-loop region. May act as a chaperone that protects the 12S mt-rRNA on the 28S mitoribosomal subunit during ribosomal small subunit assembly.
  • 基因功能參考文獻(xiàn)：
    1. Three women from three different families presented with signs of PS, including deafness and ovarian dysgenesis. We employed exome sequencing and identified a homozygous missense mutation in the ERAL1 gene, c.707A > T. The mutation leads to a damaging p.Asn236Ile substitution that is likely to interfere with the GTP binding capacity of the ERAL1 protein. PMID: 28449065
    2. Data show that loss of ERAL1 leads to rapid decay of nascent 12S mt-rRNA, consistent with a role as a mitochondrial RNA chaperone. PMID: 20604745
  • 相關(guān)疾?。?/div>
    Perrault syndrome 6 (PRLTS6)
  • 亞細(xì)胞定位：
    Mitochondrion matrix. Mitochondrion inner membrane; Peripheral membrane protein. Note=Localizes on the matrix side on the mitochondrial inner membrane.
  • 蛋白家族：
    TRAFAC class TrmE-Era-EngA-EngB-Septin-like GTPase superfamily, Era GTPase family
  • 數(shù)據(jù)庫鏈接：

    HGNC: 3424

    OMIM: 607435

    KEGG: hsa:26284

    STRING: 9606.ENSP00000254928

    UniGene: Hs.3426