概述

  • 产品名称Anti-SMN1+SMN2抗体
    参阅全部 SMN1+SMN2 一抗
  • 描述
    小鼠多克隆抗体to SMN1+SMN2
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Full length Human SMN2 (NP_075013.1)

  • 阳性对照
    • Human kidney lysate

性能

  • 形式Liquid
  • 存放说明Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
  • 存储溶液pH: 7.40
    Constituent: PBS
  • Concentration information loading...
  • 纯度Protein A purified
  • 克隆多克隆
  • 同种型IgG
  • 研究领域

应用

Our Abpromise guarantee covers the use of ab88979 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
WB 1/500 - 1/1000. Predicted molecular weight: 31 kDa.

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

  • Anti-SMN1+SMN2 antibody (ab88979) at 1/500 dilution + Human kidney lysate at 50 µg

    Predicted band size : 31 kDa
  • All lanes : Anti-SMN1+SMN2 antibody (ab88979) at 1/500 dilution

    Lane 1 : SMN2-transfected 293T cell lysate
    Lane 2 : untransfected 293T cell lysate

    Lysates/proteins at 25 µg per lane.


    Predicted band size : 31 kDa
    Observed band size : 31 kDa

Anti-SMN1+SMN2 antibody (ab88979)参考文献

ab88979 has not yet been referenced specifically in any publications.

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