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C16orf57 Antikörper (AbBy Fluor® 647)

This anti-C16orf57 antibody (ABIN1403760) is a Rabbit Polyclonal antibody detecting C16orf57 in WB, IF (p). Suitable for Human, Mouse, Rat.
Produktnummer ABIN1403760

Quick Overview for C16orf57 Antikörper (AbBy Fluor® 647) (ABIN1403760)

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® 647

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
  • 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

    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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