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C16orf57 anticorps (AbBy Fluor® 488)

USB1 Reactivité: Humain, Souris, Rat WB, IF (p) Hôte: Lapin Polyclonal AbBy Fluor® 488
N° du produit ABIN1403758
  • Antigène Voir toutes C16orf57 (USB1) Anticorps
    C16orf57 (USB1) (U6 SnRNA Biogenesis 1 (USB1))
    Reactivité
    • 25
    • 17
    • 17
    • 2
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    Humain, Souris, Rat
    Hôte
    • 22
    • 2
    • 1
    Lapin
    Clonalité
    • 25
    Polyclonal
    Conjugué
    • 9
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Cet anticorp C16orf57 est conjugé à/à la AbBy Fluor® 488
    Application
    • 21
    • 12
    • 4
    • 3
    • 1
    Western Blotting (WB), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
     Réactivité croisée
    Humain, Souris, Rat
    Purification
    Purified by Protein A.
    Immunogène
    KLH conjugated synthetic peptide derived from human C16orf57
    Isotype
    IgG
    Top Product
    Discover our top product USB1 Anticorps primaire
  • Indications d'application
    IF(IHC-P) 1:50-200
    Restrictions
    For Research Use only
  • Format
    Liquid
    Concentration
    1 μg/μL
    Buffer
    Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
    Agent conservateur
    ProClin
    Précaution d'utilisation
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    Stock
    -20 °C
    Stockage commentaire
    Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
    Date de péremption
    12 months
  • Antigène
    C16orf57 (USB1) (U6 SnRNA Biogenesis 1 (USB1))
    Autre désignation
    C16orf57 (USB1 Produits)
    Synonymes
    anticorps C16orf57, anticorps HVSL1, anticorps Mpn1, anticorps PN, anticorps hUsb1, anticorps C18H16orf57, anticorps AA960436, anticorps RGD1305215, anticorps c16orf57, anticorps C2H16orf57, anticorps zgc:91896, anticorps U6 snRNA biogenesis phosphodiesterase 1, anticorps U6 snRNA biogenesis 1, anticorps U6 snRNA biogenesis phosphodiesterase 1 L homeolog, anticorps USB1, anticorps Usb1, anticorps usb1.L, anticorps usb1
    Sujet

    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.

    ID gène
    79650
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