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SMN1 抗体

This anti-SMN1 antibody is a 兔 多克隆 antibody detecting SMN1 in WB 和 IHC. Suitable for 人, 小鼠 和 大鼠.
产品编号 ABIN6568829
发货至: 中国

Quick Overview for SMN1 抗体 (ABIN6568829)

抗原

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

适用

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人, 小鼠, 大鼠

宿主

  • 64
  • 10

克隆类型

  • 55
  • 19
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), Immunohistochemistry (IHC)
  • 纯化方法

    Affinity purification

    免疫原

    Recombinant protein of human SMN2

    亚型

    IgG
  • 应用备注

    WB 1:500 - 1:2000 IHC 1:50 - 1:200

    限制

    仅限研究用
  • 浓度

    1 mg/mL

    缓冲液

    Buffer: PBS with 0.02 % sodium azide, 50 % glycerol,  pH 7.3.

    储存液

    Sodium azide

    注意事项

    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

    储存条件

    -20 °C

    储存方法

    Store at -20°C. Avoid freeze / thaw cycles.
  • 抗原

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

    别名

    SMN1

    背景

    Synonyms: BCD541,Component of gems 1,Gemin 1,Gemin-1,OTTHUMP00000125198,OTTHUMP00000223567,OTTHUMP00000223568,OTTHUMP00000224066,OTTHUMP00000226924,SMA 1,SMA 2,SMA 3,SMA 4,SMA,SMA@,SMA1,SMA2,SMA3,SMA4,SMN,SMN,SMN1,SMN2,SMNT,Survival motor neuron protein,Survival of motor neuron 1,telomeric,T-BCD541

    Background: 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. While mutations in the telomeric copy are associated with spinal muscular atrophy, mutations in this gene, the centromeric copy, do not lead to disease. This gene 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 full length 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. Four transcript variants encoding distinct isoforms have been described.

    分子量

    Observed_MW: 35kDa

    Calculated_MW: 27kDa/28kDa/30kDa/31kDa

    基因ID

    6606

    UniProt

    Q16637

    途径

    Ribonucleoprotein Complex Subunit Organization
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