產(chǎn)品編號 | bs-13333R-BF488 |
英文名稱 | Rabbit Anti-Gemin 6/BF488 Conjugated antibody |
中文名稱 | BF488標(biāo)記的脊髓性肌萎縮癥蛋白Gemin6抗體 |
別 名 | FLJ23459; Gemin6; Gemin-6; Gem (nuclear organelle) associated protein 6; Gem-associated protein 6; GEMI6; GEMI6_HUMAN; Gemin-6; GEMIN6; SIP2. |
規(guī)格價(jià)格 | 100ul/2980元 購買 大包裝/詢價(jià) |
說 明 書 | 100ul |
研究領(lǐng)域 | 細(xì)胞生物 神經(jīng)生物學(xué) 表觀遺傳學(xué) |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | (predicted: Human, Mouse, Rat, Horse, Rabbit, ) |
產(chǎn)品應(yīng)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 | 19kDa |
性 狀 | Lyophilized or Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human Gemin 6 |
亞 型 | IgG |
純化方法 | affinity purified by Protein A |
儲 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol |
保存條件 | 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. |
產(chǎn)品介紹 |
background: Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by loss of motor neurons in the spinal cord. SMA is caused by deletion or loss-of-function mutations in the SMN (Survival of Motor Neuron) gene. Gemin6, the protein product of human chromosome 2p22.2, associates directly with SMN and is a part of the SMN complex containing Gemin2, Gemin3, Gemin4 and Gemin5 as well as several spliceosomal snRNP proteins. The SMN complex plays an essential role in spliceosomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing of the nucleus. The SMN complex is found in both the cytoplasm and the nucleus. The nuclear form is concentrated in subnuclear bodies called gems (Gemini of the coiled bodies). Function: The SMN complex plays an essential role in spliceosomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing in the nucleus. Subunit: Part of the core SMN complex that contains SMN1, GEMIN2/SIP1, DDX20/GEMIN3, GEMIN4, GEMIN5, GEMIN6, GEMIN7, GEMIN8 and STRAP/UNRIP. Interacts directly with GEMIN7, SNRPB, SNRPD2, SNRPD3 and SNRPE. Subcellular Location: Nucleus; nucleoplasm. Nucleus; gem. Cytoplasm. Found both in the nucleoplasm and in nuclear bodies called gems (Gemini of Cajal bodies) that are often in proximity to Cajal (coiled) bodies. Also found in the cytoplasm. Database links: UniProtKB/Swiss-Prot: Q8WXD5.1 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
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