SMN1 Rabbit pAb, Cy5.5 conjugated(bs-4628R-Cy5.5)-100ul

SMN1 Rabbit pAb, Cy5.5 conjuga

ted(bs-4628R-Cy5.5)-100ul
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  • ¥2980
  • Bioss已认证
  • bs-4628R-Cy5.5
  • 2025年09月30日
  • 产品信息以Bioss网站为准
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      100ul

    产品编号bs-4628R-Cy5.5
    英文名称SMN1 Rabbit pAb, Cy5.5 conjugated
    中文名称Cy5.5标记的运动神经元生存蛋白1抗体
    英文别名Component of gems 1; Component of gems 2; Gemin 1; Gemin-1; SMA; SMA1; SMA3; SMN; SMN_HUMAN; SMN1; SMN2; SMNC; SMNT; Survival motor neuron protein; survival of motor neuron 1, telomeric; survival of motor neuron 2, centromeric.
    产品应用Flow-Cyt=1ug/Test, IF=1:100-500

    Not yet tested in other applications.
    Optimal working dilutions must be determined by the end user.

    抗体来源Rabbit
    免疫原KLH conjugated synthetic peptide derived from human SMN1
    亚型IgG
    纯化方法affinity purified by Protein A
    克隆类型Polyclonal
    浓度1mg/ml
    储存液0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    研究领域

    Epigenetics and Nuclear Signaling > DNA / RNA > RNA Processing > Splicing

    Neuroscience > Neurology process > Neural Signal Transduction

    亚基Component of an import snRNP complex composed of KPNB1, RNUT1, SMN1 and ZNF259. Part of the core SMN complex that contains SMN1, GEMIN2/SIP1, DDX20/GEMIN3, GEMIN4, GEMIN5, GEMIN6, GEMIN7, GEMIN8 and STRAP/UNRIP. Interacts with DDX20, FBL, NOLA1, RNUT1, SYNCRIP and with several spliceosomal snRNP core Sm proteins, including SNRPB, SNRPD1, SNRPD2, SNRPD3, SNRPE and ILF3. Interacts with OSTF1, LSM10 and LSM11.
    亚细胞定位Cytoplasm. Nucleus, gem. Note=Localized in subnuclear structures next to coiled bodies, called Gemini of Cajal bodies (Gems).
    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).
    相似性Belongs to the SMN family.
    _x000D_ Contains 1 Tudor domain.
    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.
    保存条件Shipped at 4℃. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
    注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    背景资料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. [provided by RefSeq, Sep 2008]

     

    应用推荐稀释比例
    {Flow-Cyt}{1ug/Test}
    {IF}{1:100-500}

     

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