货号：05-1532

品牌：Merck Millipore

规格：100Ug

目录价：￥5053.00

市场价格：￥4295.05

会员价格：￥4042.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-SMN Antibody, clone 2B1 | 05-1532 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Specificity: This antibody recognizes SMN. View All» Molecular Weight: 35 kDa was observed; however, the calculated molecular weight is 31.849 kDa. View All» Epitope: Unknown View All» Immunogen: His-tagged recombinant protein corresponding to human SMN. View All» Clone: 2B1 View All» Isotype: IgG1κ View All» Background Information: The survival of motor neurons (SMN) protein is essential for the biogenesis of small nuclear RNA (snRNA)-ribonucleoproteins (snRNPs), the major components of the pre-mRNA splicing machinery. Though it is ubiquitously expressed, SMN deficiency causes the motor neuron degenerative disease spinal muscular atrophy (SMA). SMN deficiency has unexpected cell type-specific effects on the repertoire of snRNAs and mRNAs. It alters the stoichiometry of snRNAs and causes widespread pre-mRNA splicing defects in numerous transcripts of diverse genes, preferentially those containing a large number of introns, in SMN-deficient mouse tissues. The SMN complex plays a role in RNA metabolism and in splicing regulation. View All» Species Reactivity:

• Human

• Mouse

• Xenopus
  View All» Species Reactivity Note: Demonstrated to react with human. Predicted to react with mouse and xenopus based on sequence homology. View All» Application Notes: Immunocytochemistry Analysis: 1:500 dilution from a representative lot detected SMN in HeLa cells. View All» Control: HeLa cell lysate View All» Quality Assurance: Evaluated by Western Blot in HeLa cell lysate.Western Blot Analysis: 0.5 µg/ml of this antibody detected SMN on 10 µg of HeLa cell lysate. View All» Purification Method: Protein G purfied View All» Presentation: Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4, 150 mM NaCl) with 0.05% sodium azide. View All» Storage Conditions: Stable for 1 year at 2-8°C from date of receipt. View All» UniProt Number: Q16637 View All» Entrez Gene Number: NP_000335.1 View All» Gene Symbol:
  • SMN1

  • BCD541

  • Gemin-1

  • SMA

  • SMA1

  • SMA2

  • SMA3

  • SMA4

  • SMA@

  • SMN

  • SMNC

  • SMNT
    View All» Alternate Names:
    • Component of gems 1

    • gemin 1

    • spinal muscular atrophy (Werdnig-Hoffmann disease, Kugelberg-Welander disease)

    • survival of motor neuron 1, telomeric
      View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications:
      • Western Blotting

      • Immunocytochemistry

      • Immunoprecipitation
        View All» Entrez Gene Summary: 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] View All» UniProt Summary: FUNCTION: 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. SUBUNIT STRUCTURE: Component of an import snRNP complex composed of KPNB1, RNUT1, SMN1 and ZNF259. Part of the core SMN complex that contains SMN1, SIP1/GEMIN2, 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. SUBCELLULAR LOCATION: Cytoplasm. Nucleus › gem. Note: Localized in subnuclear structures next to coiled bodies, called Gemini of Cajal bodies (Gems). TISSUE SPECIFICITY: 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). INVOLVEMENT IN DISEASE: 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.MISCELLANEOUS: The SMN gene is present in two highly homologous and functional copies (TelSMN/SMN1 and CenSMN/SMN2). The telomeric copy of SMN gene (TelSMN/SMN1) seems to be the SMA-determining gene while the centromeric copy seems unaffected.SEQUENCE SIMILARILITIES: Belongs to the SMN family.Contains 1 Tudor domain. View All» Product Name: Anti-SMN, clone 2B1 View All» Concentration: 1 mg/mL View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Purified View All» Host: Mouse View All»
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