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C16orf57 Antikörper (AbBy Fluor® 488)

USB1 Reaktivität: Human, Maus, Ratte WB, IF (p) Wirt: Kaninchen Polyclonal AbBy Fluor® 488
Produktnummer ABIN1403758
  • Target Alle C16orf57 (USB1) Antikörper anzeigen
    C16orf57 (USB1) (U6 SnRNA Biogenesis 1 (USB1))
    Reaktivität
    • 25
    • 17
    • 17
    • 2
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    Human, Maus, Ratte
    Wirt
    • 22
    • 2
    • 1
    Kaninchen
    Klonalität
    • 25
    Polyklonal
    Konjugat
    • 9
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Dieser C16orf57 Antikörper ist konjugiert mit AbBy Fluor® 488
    Applikation
    • 21
    • 12
    • 4
    • 3
    • 1
    Western Blotting (WB), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
    Kreuzreaktivität
    Human, Maus, Ratte
    Aufreinigung
    Purified by Protein A.
    Immunogen
    KLH conjugated synthetic peptide derived from human C16orf57
    Isotyp
    IgG
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  • Applikationshinweise
    IF(IHC-P) 1:50-200
    Beschränkungen
    Nur für Forschungszwecke einsetzbar
  • Format
    Liquid
    Konzentration
    1 μg/μL
    Buffer
    Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
    Konservierungsmittel
    ProClin
    Vorsichtsmaßnahmen
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    Lagerung
    -20 °C
    Informationen zur Lagerung
    Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
    Haltbarkeit
    12 months
  • Target
    C16orf57 (USB1) (U6 SnRNA Biogenesis 1 (USB1))
    Andere Bezeichnung
    C16orf57 (USB1 Produkte)
    Synonyme
    C16orf57 antikoerper, HVSL1 antikoerper, Mpn1 antikoerper, PN antikoerper, hUsb1 antikoerper, C18H16orf57 antikoerper, AA960436 antikoerper, RGD1305215 antikoerper, c16orf57 antikoerper, C2H16orf57 antikoerper, zgc:91896 antikoerper, U6 snRNA biogenesis phosphodiesterase 1 antikoerper, U6 snRNA biogenesis 1 antikoerper, U6 snRNA biogenesis phosphodiesterase 1 L homeolog antikoerper, USB1 antikoerper, Usb1 antikoerper, usb1.L antikoerper, usb1 antikoerper
    Hintergrund

    Synonyms: Chromosome 16 open reading frame 57, CP057_HUMAN, FLJ13154, UPF0406 protein C16orf57.

    Background: Involvement in disease,Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN). PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.

    Gen-ID
    79650
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