概述

  • 产品名称Anti-SMN1+SMN2抗体
    参阅全部 SMN1+SMN2 一抗
  • 描述
    兔多克隆抗体to SMN1+SMN2
  • 经测试应用适用于: ICC/IF, WBmore details
  • 种属反应性
    与反应: Human
    预测可用于: Chimpanzee, Macaque Monkey, Gorilla, Orangutan
  • 免疫原

    Synthetic peptide conjugated to KLH derived from within residues 150 - 250 of Human SMN1+SMN2.

  • 阳性对照
    • This antibody gave a positive signal in both Human Kidney and Liver tissue lysate. It also gave a positive signal in HeLa cell line in IF/ICC.

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
  • 存储溶液pH: 7.40
    Preservative: 0.02% Sodium azide
    Constituent: PBS
    Note: Batches of this product that have a concentration < 1mg/ml may have BSA added as a stabilising agent. If you would like information about the formulation of a specific lot, please contact our scientific support team who will be happy to help.
  • Concentration information loading...
  • 纯度Immunogen affinity purified
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab124438 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
ICC/IF Use a concentration of 5 µg/ml.
WB Use a concentration of 1 µg/ml. Detects a band of approximately 31 kDa (predicted molecular weight: 31 kDa). Abcam recommends using milk as the blocking agent - 3%.

靶标

  • 功能The SMN complex plays an essential role in spliceosomal snRNP assembly in the cytoplasm and is required for pre-mRNA splicing in the nucleus. It may also play a role in the metabolism of snoRNPs.
  • 组织特异性Expressed in a wide variety of tissues. Expressed at high levels in brain, kidney and liver, moderate levels in skeletal and cardiac muscle, and low levels in fibroblasts and lymphocytes. Also seen at high levels in spinal cord. Present in osteoclasts and mononuclear cells (at protein level).
  • 疾病相关Defects in SMN1 are the cause of spinal muscular atrophy autosomal recessive type 1 (SMA1) [MIM:253300]. Spinal muscular atrophy refers to a group of neuromuscular disorders characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. Autosomal recessive forms are classified according to the age of onset, the maximum muscular activity achieved, and survivorship. The severity of the disease is mainly determined by the copy number of SMN2, a copy gene which predominantly produces exon 7-skipped transcripts and only low amount of full-length transcripts that encode for a protein identical to SMN1. Only about 4% of SMA patients bear one SMN1 copy with an intragenic mutation. SMA1 is a severe form, with onset before 6 months of age. SMA1 patients never achieve the ability to sit.
    Defects in SMN1 are the cause of spinal muscular atrophy autosomal recessive type 2 (SMA2) [MIM:253550]. SMA2 is an autosomal recessive spinal muscular atrophy of intermediate severity, with onset between 6 and 18 months. Patients do not reach the motor milestone of standing, and survive into adulthood.
    Defects in SMN1 are the cause of spinal muscular atrophy autosomal recessive type 3 (SMA3) [MIM:253400]. SMA3 is an autosomal recessive spinal muscular atrophy with onset after 18 months. SMA3 patients develop ability to stand and walk and survive into adulthood.
    Defects in SMN1 are the cause of spinal muscular atrophy autosomal recessive type 4 (SMA4) [MIM:271150]. SMA4 is an autosomal recessive spinal muscular atrophy characterized by symmetric proximal muscle weakness with onset in adulthood and slow disease progression. SMA4 patients can stand and walk.
  • 序列相似性Belongs to the SMN family.
    Contains 1 Tudor domain.
  • 细胞定位Cytoplasm. Nucleus > gem. Localized in subnuclear structures next to coiled bodies, called Gemini of Cajal bodies.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Component of gems 1 antibody
    • Component of gems 2 antibody
    • Gemin 1 antibody
    • Gemin-1 antibody
    • SMA antibody
    • SMA1 antibody
    • SMA3 antibody
    • SMN antibody
    • SMN_HUMAN antibody
    • SMN1 antibody
    • SMN2 antibody
    • SMNC antibody
    • SMNT antibody
    • Survival motor neuron protein antibody
    • survival of motor neuron 1, telomeric antibody
    • survival of motor neuron 2, centromeric antibody
    see all

Anti-SMN1+SMN2 antibody 图像

  • ICC/IF image of ab124438 stained HeLa cells. The cells were 100% methanol fixed (5 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (abab124438, 5µg/ml) overnight at +4°C. The secondary antibody (green) was ab96899, DyLight® 488 goat anti-rabbit IgG (H+L) used at a 1/250 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM. This antibody also gave a positive result in 100% methanol fixed (5 min) Hek293 and MCF7 cells at 5µg/ml, and in 4% formaldehyde fixed (10 min) HeLa and MCF7 cells at 5µg/ml.

  • All lanes : Anti-SMN1+SMN2 antibody (ab124438) at 1 µg/ml (Milk blocking - 3%)

    Lane 1 : Human kidney tissue lysate - total protein (ab30203)
    Lane 2 : Human liver tissue lysate - total protein (ab29889)

    Lysates/proteins at 25 µg per lane.

    Secondary
    Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/10000 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Predicted band size : 31 kDa
    Observed band size : 31 kDa
    Additional bands at : 44 kDa,49 kDa,50 kDa. We are unsure as to the identity of these extra bands.

    Exposure time : 3 minutes
    Abcam recommends using milk as the blocking agent - 3%. This blot was produced using a 10% Bis-tris gel under the MOPS buffer system. The gel was run at 200V for 50 minutes before being transferred onto a Nitrocellulose membrane at 30V for 70 minutes. The membrane was then blocked for an hour using 3% Milk before being incubated with ab124438 overnight at 4°C. Antibody binding was detected using an anti-rabbit antibody conjugated to HRP, and visualised using ECL development solution.

Anti-SMN1+SMN2 antibody (ab124438)参考文献

ab124438 has not yet been referenced specifically in any publications.

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