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GNE Antikörper (Alexa Fluor 488)

GNE Reaktivität: Human, Maus, Ratte WB, IF (p) Wirt: Kaninchen Polyclonal Alexa Fluor 488
Produktnummer ABIN1405216
  • Target Alle GNE Antikörper anzeigen
    GNE (Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE))
    Reaktivität
    Human, Maus, Ratte
    Wirt
    • 54
    • 1
    Kaninchen
    Klonalität
    • 54
    • 1
    Polyklonal
    Konjugat
    • 20
    • 5
    • 5
    • 5
    • 4
    • 4
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Dieser GNE Antikörper ist konjugiert mit Alexa Fluor 488
    Applikation
    • 54
    • 31
    • 20
    • 14
    • 12
    • 6
    • 1
    • 1
    • 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 GLCNE
    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
    GNE (Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase (GNE))
    Andere Bezeichnung
    GLCNE (GNE Produkte)
    Synonyme
    DMRV antikoerper, GLCNE antikoerper, IBM2 antikoerper, UAE1 antikoerper, zgc:77657 antikoerper, GNE antikoerper, MGC145505 antikoerper, NM antikoerper, Uae1 antikoerper, 2310066H07Rik antikoerper, glucosamine (UDP-N-acetyl)-2-epimerase/N-acetylmannosamine kinase antikoerper, GNE antikoerper, gne antikoerper, Gne antikoerper
    Hintergrund

    Synonyms: IBM2, Uae1, Bunctional UDP N acetylglucosamine 2 epimerase/N acetylmannosamine kinase, DMRV, ManAc kinase, N acylmannosamine kinase, NM, RP23-209M8.6, UDP GlcNAc 2 epimerase, UDP GlcNAc 2 epimerase/ManAc kinase, Uridine diphosphate N acetylglucosamine 2 epimerase, GLCNE_HUMAN.

    Background: The bifunctional enzyme UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase (GNE/Mnk), or GLCNE, regulates and initiates biosynthesis of N-acetylneuraminic acid (NeuAc), a precursor of sialic acids. GLCNE is required for normal sialylation in hematopoietic cells. Sialylation is implicated in cell adhesion, signal transduction, tumorigenicity and metastatic behavior of malignant cells. It is upregulated after PKC-dependent phosphorylation and is most abundantly expressed in liver and placenta. It is also expressed, to a lesser extent, in heart, brain, lung, kidney, skeletal muscle and pancreas. Defects in GLCNE are the cause of sialuria, inclusion body myopathy 2 (IBM2) and Nonaka myopathy (NM) or distal myopathy with rimmed vacuoles (DMRV). Sialuria is an autosomal dominant disorder caused by a lack of feedback inhibition of GLCNE by CMP-NeuAc, resulting in overproduction of NeuAc. It is characterized by an accumulation of free sialic acid in the cytoplasm and large quantities of neuraminic acid in the urine. Both IBM2 and NM/DMRV are autosomal recessive neuromuscular disorders characterized by adult onset, distal and proximal muscle weakness (especially in the legs) and a typical muscle pathology including filamentous inclusions and rimmed vacuoles.

    Gen-ID
    10020
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