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SMN1 抗体

This anti-SMN1 antibody is a 兔 多克隆 antibody detecting SMN1 in WB, IF 和 ICC. Suitable for 人. This Primary Antibody has been cited in 1 publication.
产品编号 ABIN2854909
发货至: 中国

Quick Overview for SMN1 抗体 (ABIN2854909)

抗原

See all SMN1 抗体
SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))

适用

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宿主

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克隆类型

  • 55
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多克隆

标记

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This SMN1 antibody is un-conjugated

应用范围

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Western Blotting (WB), Immunofluorescence (IF), Immunocytochemistry (ICC)
  • 交叉反应

    产品特性

    Rabbit Polyclonal antibody to SMN1 (survival of motor neuron 1, telomeric)
    SMN1 antibody

    纯化方法

    Purified by antigen-affinity chromatography.

    免疫原

    Recombinant protein encompassing a sequence within the center region of human SMN1. The exact sequence is proprietary.

    亚型

    IgG
  • 应用备注

    WB: 1:500-1:3000. ICC/IF: 1:100-1:1000. Optimal dilutions/concentrations should be determined by the researcher. Not tested in other applications.

    说明

    Positive Control: 293T , A431 , HeLa , HepG2

    限制

    仅限研究用
  • 状态

    Liquid

    浓度

    1 mg/mL

    缓冲液

    1XPBS ( pH 7), 40 % Glycerol, 0.01 % Thimerosal

    储存液

    Thimerosal (Merthiolate)

    注意事项

    This product contains Thimerosal (Merthiolate): a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

    储存条件

    4 °C,-20 °C

    储存方法

    Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4°C. For long-term storage, aliquot and store at -20°C or below. Avoid multiple freeze-thaw cycles.
  • Zhang, Lin, Li, Lu, Guo, Dong, Zhao, He, Wang, Chen: "Application of urine cells in drug intervention for spinal muscular atrophy." in: Experimental and therapeutic medicine, Vol. 14, Issue 3, pp. 1993-1998, (2017) (PubMed).

  • 抗原

    SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))

    别名

    survival of motor neuron 1, telomeric

    背景

    This gene is part of a 500 kb inverted duplication on chromosome 5q13. This duplicated region contains at least four genes and repetitive elements which make it prone to rearrangements and deletions. The repetitiveness and complexity of the sequence have also caused difficulty in determining the organization of this genomic region. The telomeric and centromeric copies of this gene are nearly identical and encode the same protein. However, mutations in this gene, the telomeric copy, are associated with spinal muscular atrophy, mutations in the centromeric copy do not lead to disease. The centromeric copy may be a modifier of disease caused by mutation in the telomeric copy. The critical sequence difference between the two genes is a single nucleotide in exon 7, which is thought to be an exon splice enhancer. Note that the nine exons of both the telomeric and centromeric copies are designated historically as exon 1, 2a, 2b, and 3-8. It is thought that gene conversion events may involve the two genes, leading to varying copy numbers of each gene. The protein encoded by this gene localizes to both the cytoplasm and the nucleus. Within the nucleus, the protein localizes to subnuclear bodies called gems which are found near coiled bodies containing high concentrations of small ribonucleoproteins (snRNPs). This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, and also interacts with several proteins known to be involved in the biogenesis of snRNPs, such as hnRNP U protein and the small nucleolar RNA binding protein. Two transcript variants encoding distinct isoforms have been described.

    分子量

    32 kDa

    基因ID

    6606

    UniProt

    Q16637

    途径

    Ribonucleoprotein Complex Subunit Organization
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