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SMN1 / SMN2 抗体

适用: 人 WB, IHC (p), ICC 宿主: 小鼠 Monoclonal 2B10 unconjugated
产品编号 ABIN6654200
发货至: 中国
  • 抗原
    SMN1 / SMN2
    适用
    • 5
    • 1
    • 1
    宿主
    • 2
    • 2
    • 1
    小鼠
    克隆类型
    • 3
    • 2
    单克隆
    标记
    • 5
    非结合性
    应用范围
    • 5
    • 1
    • 1
    • 1
    • 1
    • 1
    Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunocytochemistry (ICC)
    纯化方法
    Protein G affinity
    免疫原
    Amino acids RRGTGQSDDSDIWDDTALIKAYDKAVASFKH were used as the immunogen for the SMN1/2 antibody.
    克隆位点
    2B10
    亚型
    IgG1
  • 应用备注
    Optimal dilution of the SMN1/2 antibody should be determined by the researcher.\. Western blot: 0.5-1 μg/mL,IHC (FFPE): 0.5-1 μg/mL,ICC: 0.5-1 μg/mL
    限制
    仅限研究用
  • 缓冲液
    0.5 mg/mL if reconstituted with 0.2 mL sterile DI water
    储存条件
    -20 °C
    储存方法
    After reconstitution, the SMN1/2 antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.
  • 抗原
    SMN1 / SMN2
    别名
    SMN1/2
    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. Multiple transcript variants encoding distinct isoforms have been described.
    UniProt
    Q16637
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