Recombinant
RabMAb

Recombinant Anti-GNE antibody [EPR15059] - C-terminal (ab189927)

Overview

  • Product name

    Anti-GNE antibody [EPR15059] - C-terminal
    See all GNE primary antibodies
  • Description

    Rabbit monoclonal [EPR15059] to GNE - C-terminal
  • Host species

    Rabbit
  • Tested applications

    Suitable for: ICC/IF, IP, WBmore details
  • Species reactivity

    Reacts with: Rat, Human
  • Immunogen

    within Human GNE aa 700 to the C-terminus. The exact sequence is proprietary.
    Database link: Q9Y223

  • Positive control

    • Human fetal brain, fetal kidney, fetal liver, HeLa, A549 and K562 lysates; HeLa cells.
  • General notes

     This product was previously labelled as GLCNE

     

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    This product is a recombinant rabbit monoclonal antibody.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer

    Preservative: 0.01% Sodium azide
    Constituents: 59% PBS, 40% Glycerol, 0.05% BSA
  • Concentration information loading...
  • Purity

    Protein A purified
  • Clonality

    Monoclonal
  • Clone number

    EPR15059
  • Isotype

    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab189927 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
ICC/IF 1/500.
IP 1/30 - 1/40.
WB 1/1000 - 1/10000. Detects a band of approximately 75 kDa (predicted molecular weight: 79 kDa).

Target

  • Function

    Regulates and initiates biosynthesis of N-acetylneuraminic acid (NeuAc), a precursor of sialic acids. Plays an essential role in early development (By similarity). Required for normal sialylation in hematopoietic cells. Sialylation is implicated in cell adhesion, signal transduction, tumorigenicity and metastatic behavior of malignant cells.
  • Tissue specificity

    Highest expression in liver and placenta. Also found in heart, brain, lung, kidney, skeletal muscle and pancreas. Isoform 1 is expressed in heart, brain, kidney, liver, placenta, lung, spleen, pancreas, skeletal muscle and colon. Isoform 2 is expressed mainly in placenta, but also in brain, kidney, liver, lung, pancreas and colon. Isoform 3 is expressed at low level in kidney, liver, placenta and colon.
  • Pathway

    Amino-sugar metabolism; N-acetylneuraminate biosynthesis.
  • Involvement in disease

    Defects in GNE are a cause of sialuria (SIALURIA) [MIM:269921]; also known as sialuria French type. In sialuria, free sialic acid accumulates in the cytoplasm and gram quantities of neuraminic acid are secreted in the urine. The metabolic defect involves lack of feedback inhibition of UDP-GlcNAc 2-epimerase by CMP-Neu5Ac, resulting in constitutive overproduction of free Neu5Ac. Clinical features include variable degrees of developmental delay, coarse facial features and hepatomegaly. Sialuria inheritance is autosomal dominant.
    Defects in GNE are the cause of inclusion body myopathy type 2 (IBM2) [MIM:600737]. Hereditary inclusion body myopathies are a group of neuromuscular disorders characterized by adult onset, slowly progressive distal and proximal weakness and a typical muscle pathology including rimmed vacuoles and filamentous inclusions. IBM2 is an autosomal recessive disorder affecting mainly leg muscles, but with an unusual distribution that spares the quadriceps as also observed in Nonaka myopathy.
    Defects in GNE are the cause of Nonaka myopathy (NM) [MIM:605820]; also known as distal myopathy with rimmed vacuoles (DMRV). NM is an autosomal recessive muscular disorder, allelic to inclusion body myopathy 2. It is characterized by weakness of the anterior compartment of the lower limbs with onset in early adulthood, and sparing of the quadriceps muscles. As the inclusion body myopathy, NM is histologically characterized by the presence of numerous rimmed vacuoles without inflammatory changes in muscle specimens.
  • Sequence similarities

    In the N-terminal section; belongs to the UDP-N-acetylglucosamine 2-epimerase family.
    In the C-terminal section; belongs to the ROK (NagC/XylR) family.
  • Post-translational
    modifications

    Phosphorylated by PKC.
  • Cellular localization

    Cytoplasm.
  • Information by UniProt
  • Database links

  • Alternative names

    • 2310066H07Rik antibody
    • Bifunctional UDP N acetylglucosamine 2 epimerase/N acetylmannosamine kinase antibody
    • DMRV antibody
    • GLCNE_HUMAN antibody
    • Glucosamine (UDP N acetyl) 2 epimerase/N acetylmannosamine kinase antibody
    • GNE antibody
    • IBM2 antibody
    • ManAc kinase antibody
    • N acylmannosamine kinase antibody
    • N-acetylmannosamine kinase antibody
    • NM antibody
    • RP23-209M8.6 antibody
    • Uae1 antibody
    • UDP GlcNAc 2 epimerase antibody
    • UDP GlcNAc 2 epimerase/ManAc kinase antibody
    • UDP N acetylglucosamine 2 epimerase/N acetylmannosamine kinase antibody
    • UDP-GlcNAc-2-epimerase antibody
    • UDP-GlcNAc-2-epimerase/ManAc kinase antibody
    • Uridine diphosphate N acetylglucosamine 2 epimerase antibody
    • Uridine diphosphate-N-acetylglucosamine-2-epimerase antibody
    see all

Images

  • All lanes : Anti-GNE antibody [EPR15059] - C-terminal (ab189927) at 1/1000 dilution

    Lane 1 : Human fetal liver
    Lane 2 : Human fetal kidney
    Lane 3 : Human fetal brain

    Lysates/proteins at 20 µg per lane.

    Secondary
    All lanes : Anti-Rabbit IgG (HRP), specific to the non-reduced form of IgG at 1/1000 dilution

    Predicted band size: 79 kDa
    Observed band size: 75 kDa
    why is the actual band size different from the predicted?

  • All lanes : Anti-GNE antibody [EPR15059] - C-terminal (ab189927) at 1/10000 dilution

    Lane 1 : HeLa cell lysate
    Lane 2 : A549 cell lysate
    Lane 3 : K562 cell lysate

    Lysates/proteins at 20 µg per lane.

    Secondary
    All lanes : Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugate at 1/1000 dilution

    Predicted band size: 79 kDa
    Observed band size: 75 kDa why is the actual band size different from the predicted?

  • Immunofluorescent analysis of 4% paraformaldehyde-fixed HeLa cells labeling GNE with ab189927 at 1/500 dilution followed by Goat anti rabbit IgG (Alexa Fluor® 555) secondary antibody at 1/200 dilution.  Counter stained with DAPI.

  • Western blot analysis of GNE in A549 cell lysate immunoprecipitated using ab189927 at 1/50 dilution.

    Secondary antibody: Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugate at 1/1000 dilution.

References

ab189927 has not yet been referenced specifically in any publications.

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