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MABE230 Anti-SMN2 Antibody, clone SMN-KH

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      Key Specifications Table

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      HWB, IHCMPurifiedMonoclonal Antibody
      Catalogue NumberMABE230
      DescriptionAnti-SMN2 Antibody, clone SMN-KH
      Alternate Names
      • Survival motor neuron protein
      • Component of gems 1
      • Gemin-1
      Background InformationSurvival of Motor Neuron (SMN) is expressed from two linked paralogous genes, SMN1 and SMN2. SMNs are primarily localized in the cytoplasm and nuclear gems of all cells, where they are understood to mediate the assembly of spliceosomal small nuclear ribonucleoprotein particles (snRNPs). They also play a role in the metabolism of snoRNPs and are required for the splicing of pre-mRNA in the nucleus. The progressive loss of functional SMNs in the anterior horn of the spinal cord is a critical cause of Spinal Muscular Atrophy (SMA), an autosomal recessive neuromuscular disease. There are three known types of childhood-onset SMA, in addition to a fourth type that is characterized by adult-onset SMA.
      Product Information
      • HeLa cell lysate
      PresentationPurified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
      Quality LevelMQ100
      ApplicationAnti-SMN2 Antibody, clone SMN-KH is a Mouse Monoclonal Antibody for detection of SMN2 also known as Survival motor neuron protein & has been validated in WB & IHC.
      Key Applications
      • Western Blotting
      • Immunohistochemistry
      Application NotesWestern Blot Analysis: A representative lot from an independent laboratory detected SMN2 in HeLa cell lysate.

      Immunohistochemistry Analysis: A respresentative lot from an independent laboratory detected human SMN2 in SMA type III mouse model thoracic spinal cord tissue. (Hua, Y., et al. (2010). Genes Dev. 24(15):1634-1644.)
      Biological Information
      ImmunogenMBP-tagged recombinant protein corresponding to human SMN2.
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      Species Reactivity
      • Human
      Antibody TypeMonoclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThis 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.
      Gene Symbol
      • SMN1
      • SMN
      • SMNT
      • SMN2
      • SMNC
      Purification MethodProtein G Purified
      UniProt Number
      UniProt SummaryFUNCTION: 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, 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.

      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).

      INVLOVMENT IN DISEASE: Defects in SMN1 are the cause of spinal muscular atrophy autosomal recessive type 1 (SMA1). 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). 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). 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). 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 SIMILARITIES: Belongs to the SMN family. Contains 1 Tudor domain.
      Molecular Weight~35 kDa observed
      Physicochemical Information
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated by Western Blot in HeLa cell lysate.

      Western Blot Analysis: 0.5 µg/mL of this antibody detected SMN2 in 10 µg of HeLa cell lysate.
      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.
      Storage and Shipping Information
      Storage ConditionsStable for 1 year at 2-8°C from date of receipt.
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information


      Anti-SMN2 Antibody, clone SMN-KH SDS


      Safety Data Sheet (SDS) 

      Anti-SMN2 Antibody, clone SMN-KH Certificates of Analysis

      TitleLot Number
      Anti-SMN2, clone SMN-KH 2476784
      Anti-SMN2, clone SMN-KH - 2227167 2227167
      Anti-SMN2, clone SMN-KH - 2298452 2298452
      Anti-SMN2, clone SMN-KH - 3245630 3245630
      Anti-SMN2, clone SMN-KH - 3420896 3420896
      Anti-SMN2, clone SMN-KH - 3439700 3439700
      Anti-SMN2, clone SMN-KH - 3492718 3492718
      Anti-SMN2, clone SMN-KH - 3574551 3574551
      Anti-SMN2, clone SMN-KH - 3714429 3714429
      Anti-SMN2, clone SMN-KH - 3772435 3772435


      Reference overviewPub Med ID
      Antisense correction of SMN2 splicing in the CNS rescues necrosis in a type III SMA mouse model.
      Hua, Yimin, et al.
      Genes Dev., 24: 1634-44 (2010)  2010

      Show Abstract
      20624852 20624852

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      Life Science Research > Antibodies and Assays > Primary Antibodies