Millipore Sigma Vibrant Logo

05-1532 Anti-SMN Antibody, clone 2B1

Anti-SMN Antibody, clone 2B1 is a Mouse Monoclonal Antibody for detection of SMN also known as Spinal muscular atrophy (Werdnig-Hoffmann disease Kugelberg-Welander disease) & has been validated in WB, ICC & IP.

Anti-SMN Antibody, clone 2B1 : FDS (Fiches de données de sécurité), certificats d’analyse (CoA) et de qualité (CoQ), dossiers, brochures et autres documents disponibles.
Research Category: Epigenetics & Nuclear Function Research Sub-Category: RNA Metabolism & Binding Proteins, RNA Metabolism & Binding Proteins Gene Symbol: SMN1, BCD541, Gemin-1, SMA, SMA1, SMA2, SMA3, SMA4, SMA@, SMN, SMNC, SMNT UniProt Number: Q16637
View Products on Sigmaaldrich.com
05-1532
100 µg  
Prix en cours de récupération
Le prix n'a pas pu être récupéré
La quantité minimale doit être un multiple de
Maximum Quantity is
À la validation de la commande Plus d'informations
Vous avez sauvegardé ()
 
—
Demander le prix
—
Disponibilité limitée Disponibilité limitée
En stock 
Interrompu(e)
Disponible en quantités limitées
Disponibilité à confirmer
    Pour le restant : Nous vous tiendrons informé
      Pour le restant : Nous vous tiendrons informé
      Nous vous tiendrons informé
      Contacter le Service Clients
      Contact Customer Service

      Offres spéciales

       

      Contacter le Service Clients

      Aperçu

      Replacement Information

      Offres spéciales

      100% Performance Guaranteed

      Tableau de caractéristiques principal

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      H, M, XnWB, ICC, IPMPurifiedMonoclonal Antibody
      Description
      Catalogue Number05-1532
      DescriptionAnti-SMN Antibody, clone 2B1
      Alternate Names
      • Component of gems 1
      • gemin 1
      • spinal muscular atrophy (Werdnig-Hoffmann disease, Kugelberg-Welander disease)
      • survival of motor neuron 1, telomeric
      Background InformationThe 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.
      References
      Product Information
      FormatPurified
      Control
      • 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
      Applications
      ApplicationAnti-SMN Antibody, clone 2B1 is a Mouse Monoclonal Antibody for detection of SMN also known as Spinal muscular atrophy (Werdnig-Hoffmann disease Kugelberg-Welander disease) & has been validated in WB, ICC & IP.
      Key Applications
      • Western Blotting
      • Immunocytochemistry
      • Immunoprecipitation
      Application NotesImmunocytochemistry Analysis: 1:500 dilution from a representative lot detected SMN in HeLa cells.
      Biological Information
      ImmunogenHis-tagged recombinant protein corresponding to human SMN.
      EpitopeUnknown
      Clone2B1
      ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
      HostMouse
      SpecificityThis antibody recognizes SMN.
      IsotypeIgG1κ
      Species Reactivity
      • Human
      • Mouse
      • Xenopus
      Species Reactivity NoteDemonstrated to react with human. Predicted to react with mouse and xenopus based on sequence homology.
      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. [provided by RefSeq]
      Gene Symbol
      • SMN1
      • BCD541
      • Gemin-1
      • SMA
      • SMA1
      • SMA2
      • SMA3
      • SMA4
      • SMA@
      • SMN
      • SMNC
      • SMNT
      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, 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.
      Molecular Weight 35 kDa was observed; however, the calculated molecular weight is 31.849 kDa.
      Physicochemical Information
      Dimensions
      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 SMN on 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
      Specifications
      Global Trade Item Number
      Référence GTIN
      05-1532 04053252741173

      Documentation

      Anti-SMN Antibody, clone 2B1 FDS

      Titre

      Fiche de données de sécurité des matériaux (FDS) 

      Anti-SMN Antibody, clone 2B1 Certificats d'analyse

      TitreNuméro de lot
      Anti-SMN, clone 2B1 2470958
      Anti-SMN, clone 2B1 - 2006960 2006960
      Anti-SMN, clone 2B1 - 2189741 2189741
      Anti-SMN, clone 2B1 - 2262736 2262736
      Anti-SMN, clone 2B1 - 2364201 2364201
      Anti-SMN, clone 2B1 - 3239899 3239899
      Anti-SMN, clone 2B1 - 3489750 3489750
      Anti-SMN, clone 2B1 - 3585808 3585808
      Anti-SMN, clone 2B1 - 3705804 3705804
      Anti-SMN, clone 2B1 - 3807942 3807942
      Show more

      Références bibliographiques

      Aperçu de la référence bibliographiqueNº PubMed
      Autoantibodies to survival of motor neuron complex in patients with polymyositis: immunoprecipitation of D, E, F, and G proteins without other components of small nuclear ribonucleoproteins.
      Satoh, Minoru, et al.
      Arthritis Rheum., 63: 1972-8 (2011)  2010

      Afficher le résumé       		21425128 21425128

      Produits & Applications associés

      Related Products

      Référence Description  
      03-178 RIPAb+ SMN - RIP Validated Antibody and Primer Set Prix & Disponibilité
      03-200 RIPAb+ SMN - RIP Validated Antibody and Primer Set Prix & Disponibilité
      04-1466 Anti-SMN Antibody, clone 62E7 Prix & Disponibilité
      MABE230 Anti-SMN2 Antibody, clone SMN-KH Prix & Disponibilité

      Catégories

      Life Science Research > Antibodies and Assays > Primary Antibodies