Anti-GLCNE antibody - N-terminal (ab170550)

Overview

  • Product name
    Anti-GLCNE antibody - N-terminal
    See all GLCNE primary antibodies
  • Description
    Rabbit polyclonal to GLCNE - N-terminal
  • Tested applications
    Suitable for: WB, IHC-Pmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Synthetic peptide within Human GLCNE aa 168-197 (N terminal) conjugated to Keyhole Limpet Haemocyanin (KLH). The exact sequence is proprietary.
    Database link: Q9Y223

  • Positive control
    • Human placenta lysate; Human liver tissue.

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: 0.09% Sodium azide
    Constituent: 99% PBS
  • Concentration information loading...
  • Purity
    Immunogen affinity purified
  • Clonality
    Polyclonal
  • Isotype
    IgG
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab170550 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
WB 1/100 - 1/500. Predicted molecular weight: 79 kDa.
IHC-P 1/10 - 1/50. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

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

  • Immunohistochemical analysis of formalin fixed, paraffin embedded Human liver tissue labeling GLCNE with ab170550 at 1/10 followed by peroxidase conjugation of the secondary antibody and DAB staining

  • Anti-GLCNE antibody - N-terminal (ab170550) at 1/100 dilution + Human placenta lysate at 35 µg

    Secondary
    Anti-rabbit HRP conjugated antibody at 1/10000 dilution
    Developed using the ECL technique

    Predicted band size : 79 kDa

References

ab170550 has not yet been referenced specifically in any publications.

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