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SMN1 Antikörper

Dieses Kaninchen Polyklonal-Antikörper erkennt spezifisch SMN1 in WB, IF und ICC. Er zeigt eine Reaktivität gegenüber Human und wurde in 1 Publikation erwähnt.
Produktnummer ABIN2854909

Kurzübersicht für SMN1 Antikörper (ABIN2854909)

Target

Alle SMN1 Antikörper anzeigen
SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))

Reaktivität

  • 59
  • 33
  • 16
  • 6
  • 6
  • 3
  • 3
  • 2
  • 2
  • 1
  • 1
  • 1
Human

Wirt

  • 64
  • 10
Kaninchen

Klonalität

  • 55
  • 19
Polyklonal

Konjugat

  • 36
  • 5
  • 4
  • 3
  • 2
  • 2
  • 2
  • 2
  • 2
  • 2
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
Dieser SMN1 Antikörper ist unkonjugiert

Applikation

  • 54
  • 27
  • 14
  • 13
  • 13
  • 10
  • 8
  • 8
  • 7
  • 3
  • 3
  • 1
  • 1
Western Blotting (WB), Immunofluorescence (IF), Immunocytochemistry (ICC)
  • Kreuzreaktivität

    Human

    Produktmerkmale

    Rabbit Polyclonal antibody to SMN1 (survival of motor neuron 1, telomeric)
    SMN1 antibody

    Aufreinigung

    Purified by antigen-affinity chromatography.

    Immunogen

    Recombinant protein encompassing a sequence within the center region of human SMN1. The exact sequence is proprietary.

    Isotyp

    IgG
  • Applikationshinweise

    WB: 1:500-1:3000. ICC/IF: 1:100-1:1000. Optimal dilutions/concentrations should be determined by the researcher. Not tested in other applications.

    Kommentare

    Positive Control: 293T , A431 , HeLa , HepG2

    Beschränkungen

    Nur für Forschungszwecke einsetzbar
  • Format

    Liquid

    Konzentration

    1 mg/mL

    Buffer

    1XPBS ( pH 7), 40 % Glycerol, 0.01 % Thimerosal

    Konservierungsmittel

    Thimerosal (Merthiolate)

    Vorsichtsmaßnahmen

    This product contains Thimerosal (Merthiolate): a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

    Lagerung

    4 °C,-20 °C

    Informationen zur Lagerung

    Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4°C. For long-term storage, aliquot and store at -20°C or below. Avoid multiple freeze-thaw cycles.
  • Zhang, Lin, Li, Lu, Guo, Dong, Zhao, He, Wang, Chen: "Application of urine cells in drug intervention for spinal muscular atrophy." in: Experimental and therapeutic medicine, Vol. 14, Issue 3, pp. 1993-1998, (2017) (PubMed).

  • Target

    SMN1 (Survival of Motor Neuron 1, Telomeric (SMN1))

    Andere Bezeichnung

    survival of motor neuron 1, telomeric

    Hintergrund

    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.

    Molekulargewicht

    32 kDa

    Gen-ID

    6606

    UniProt

    Q16637

    Pathways

    Ribonucleoprotein Complex Subunit Organization
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