ERAL1 Recombinant Monoclonal Antibody
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中文名稱：ERAL1 Recombinant Monoclonal Antibody
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貨號：CSB-RA007759MA1HU
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規格：￥1320
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圖片：
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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.
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其他：
產品詳情
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產品描述：ERAL1 Recombinant Monoclonal Antibody（CSB-RA007759MA1HU）是一款針對線粒體核糖體組裝因子ERAL1靶點開發的高特異性抗體。ERAL1作為線粒體核糖體小亞基的伴侶蛋白，在維持線粒體RNA穩定性及核糖體組裝中發揮關鍵作用，其表達異常與能量代謝紊亂及細胞凋亡調控密切相關。本產品經嚴格驗證，可精準識別內源性ERAL1蛋白，適用于ELISA和免疫組化（IHC）等實驗，其中IHC推薦使用稀釋比例為1:50-1:200，實驗數據顯示其在多種組織樣本中呈現清晰的定位信號與低背景干擾。該抗體適用于探究線粒體功能調控機制、細胞代謝相關通路研究以及疾病模型中ERAL1表達水平變化的分析，為解析線粒體相關疾病的分子機制提供可靠工具。其穩定性和重復性已通過多批次質檢驗證，適用于基礎科研領域中對線粒體核糖體組裝、RNA加工及能量代謝過程的分子生物學研究。
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Uniprot No.：
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基因名：ERAL1
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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
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反應種屬：Human
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免疫原：Recombinant Human ERAL1 protein
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
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克隆類型：Monoclonal
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抗體亞型：hIgG1
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純化方式：Affinity-chromatography
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克隆號：27A9
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 -
產品提供形式：Liquid
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應用范圍：ELISA, IHC
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推薦稀釋比：
Application Recommended Dilution IHC 1:50-1:200 -
Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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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.
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基因功能參考文獻：
- 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
- 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
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相關疾病：Perrault syndrome 6 (PRLTS6)
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亞細胞定位：Mitochondrion matrix. Mitochondrion inner membrane; Peripheral membrane protein. Note=Localizes on the matrix side on the mitochondrial inner membrane.
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蛋白家族：TRAFAC class TrmE-Era-EngA-EngB-Septin-like GTPase superfamily, Era GTPase family
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數據庫鏈接：
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