Date published: 2025-9-8

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SMN 抗体 (H-7): sc-137200

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说明书
  • SMN抗体(H-7)是小鼠单克隆IgM κ, 在1篇文献中引用,规格为200 µg/ml
  • 免疫原氨基酸序列1-195位于human物种的SMN的N-terminus附近
  • 推荐用于 human 来源的SMN WB, IP, IF 和 ELISA检测
  • 看 SMN (2B1): sc-32313,可查看有标记的 SMN 抗体,包括AC, HRP, FITC, PE, Alexa Fluor® 488, 594, 647, 680 和 790
  • m-IgGκ BP-HRP是SMN Antibody (H-7) 适用于 WB 应用。 的首选辅助检测试剂。该试剂现可与SMN Antibody (H-7) 搭配使用(请参阅下面的订购信息)。有关其它 m-IgGκ BP 结合物,请参阅我们完整的小鼠 IgG 结合蛋白列表。

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関連項目

脊髓性肌萎缩症(SMA)是一种常染色体隐性神经退行性疾病,以脊髓运动神经元丧失为特征。SMA是由SMN(运动神经元生存)基因的缺失或功能丧失突变引起的。SMN,也称为Gemin1、SMN1、SMNT和BCD541,以通过选择性剪接产生的四种异构体形式存在。SMN是多聚体,与Gemin2(前SIP1)、Gemin3(DEAD box RNA解旋酶)、Gemin4、Gemin5和Gemin6以及几种剪接体snRNP蛋白质形成复合物。SMN复合物在细胞质中的剪接体snRNP组装中起着至关重要的作用,并且是细胞核前mRNA剪接所必需的。SMN复合物既存在于细胞质中,也存在于细胞核中。核型集中在称为gems(卷曲体的双子座)的亚核体中。细胞质SMN与剪接体Sm蛋白质相互作用并促进其组装到U snRNAs上,而核SMN则介导前mRNA剪接因子的再循环。几乎相同的端粒和着丝粒形式的SMN编码相同的蛋白质;然而,只有端粒形式的突变才与疾病状态SMA相关。SMN在大脑、肾脏、肝脏、脊髓以及骨骼肌和心肌中均有广泛表达。

仅限研究使用。不适用于诊断和治疗用途。

Alexa Fluor® 是Molecular Probes Inc., OR., USA的商标

LI-COR®和 Odyssey® 是LI-COR Biosciences的注册商标

SMN 抗体 (H-7) 参考文献:

  1. SMN 复合物:RNPs 的组装机。  |  Battle, DJ., et al. 2006. Cold Spring Harb Symp Quant Biol. 71: 313-20. PMID: 17381311
  2. SMN 复合物的分子功能  |  Kolb, SJ., et al. 2007. J Child Neurol. 22: 990-4. PMID: 17761654
  3. 脊髓性肌萎缩症和 snRNP 生物发生中的 SMN。  |  Coady, TH. and Lorson, CL. 2011. Wiley Interdiscip Rev RNA. 2: 546-64. PMID: 21957043
  4. 脊髓性肌肉萎缩症:SMN 在轴突 mRNA 调节中的作用。  |  Fallini, C., et al. 2012. Brain Res. 1462: 81-92. PMID: 22330725
  5. 用于治疗脊髓性肌萎缩症的 SMN 诱导化合物。  |  Lorson, MA. and Lorson, CL. 2012. Future Med Chem. 4: 2067-84. PMID: 23157239
  6. SMN通过miR-183/mTOR途径调节轴突局部翻译。  |  Kye, MJ., et al. 2014. Hum Mol Genet. 23: 6318-31. PMID: 25055867
  7. SMN 表达的发育调控:对脊髓性肌萎缩症的病理生理学影响和疗法开发前景。  |  Jablonka, S. and Sendtner, M. 2017. Gene Ther. 24: 506-513. PMID: 28556834
  8. 存活运动神经元蛋白(SMN)在蛋白质平衡中的作用。  |  Chaytow, H., et al. 2018. Cell Mol Life Sci. 75: 3877-3894. PMID: 29872871
  9. 组合方法可提高 SMA 模型小鼠的 SMN 水平。  |  Dumas, SA., et al. 2022. Hum Mol Genet. 31: 2989-3000. PMID: 35419606
  10. 腺相关病毒 SMN 基因治疗后的致命血栓性微血管病病例。  |  Guillou, J., et al. 2022. Blood Adv. 6: 4266-4270. PMID: 35584395

订购信息

产品名称产品编号规格价格数量收藏夹

SMN 抗体 (H-7)

sc-137200
200 µg/ml
RMB2377.00

SMN (H-7): m-IgGκ BP-HRP 套装

sc-550739
200 µg Ab; 40 µg BP
RMB2662.00

What is the recommended fixation method for immunofluorescence staining with SMN (H-7): sc-137200 monoclonal antibody?

Asked by: TinTin
Thank you for your inquiry. We recommend fixing cells for 5 minutes in -10°C methanol and allowing to air dry. The full protocol can be viewed here: https://www.scbt.com/scbt/resources/protocols/immunofluorescence-cell-staining
Answered by: Technical Support
Date published: 2017-03-01
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Rated 5 out of 5 by from Good specificity in WB in HeLa whole cell lysateGood specificity in WB in HeLa whole cell lysate. -SCBT QC
Date published: 2015-07-13
Rated 5 out of 5 by from Robust signal in WB in human transfectedRobust signal in WB in human transfected 293T and HeLa whole cell lysates. -SCBT QC
Date published: 2015-04-28
Rated 5 out of 5 by from Strong band in WB in 293T whole cell lysate and KStrong band in WB in 293T whole cell lysate and K-562 nuclear extract. -SCBT QC
Date published: 2015-02-17
Rated 5 out of 5 by from Exceptional immunofluorescence cytoplasmicExceptional immunofluorescence cytoplasmic and nuclear staining in methanol-fixed HeLa cells. -SCBT QC
Date published: 2015-01-16
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SMN 抗体 (H-7) is rated 5.0 out of 5 by 4.
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