Anti-GNE antibody (ab244403)
Key features and details
- Rabbit polyclonal to GNE
- Suitable for: ICC/IF, WB, IHC-P
- Reacts with: Human
- Isotype: IgG
Overview
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Product name
Anti-GNE antibody
See all GNE primary antibodies -
Description
Rabbit polyclonal to GNE -
Host species
Rabbit -
Tested applications
Suitable for: ICC/IF, WB, IHC-Pmore details -
Species reactivity
Reacts with: Human
Predicted to work with: Mouse, Rat, Chinese hamster -
Immunogen
Recombinant fragment corresponding to Human GNE aa 250-450.
Database link: Q9Y223 -
Positive control
- ICC/IF: A431 cells. WB: A549 cell lysate. IHC-P: Human liver tissue.
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General notes
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
Properties
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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
pH: 7.20
Preservative: 0.02% Sodium azide
Constituents: PBS, 40% Glycerol (glycerin, glycerine) -
Concentration information loading...
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Purity
Immunogen affinity purified -
Clonality
Polyclonal -
Isotype
IgG -
Research areas
Associated products
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Compatible Secondaries
Applications
The Abpromise guarantee
Our Abpromise guarantee covers the use of ab244403 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 |
Use a concentration of 0.25 - 2 µg/ml.
Fixation/Permeabilization: PFA/Triton X-100. |
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WB |
Use a concentration of 0.04 - 0.4 µg/ml.
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IHC-P |
1/50 - 1/200. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
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Notes |
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ICC/IF
Use a concentration of 0.25 - 2 µg/ml. Fixation/Permeabilization: PFA/Triton X-100. |
WB
Use a concentration of 0.04 - 0.4 µg/ml. |
IHC-P
1/50 - 1/200. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol. |
Target
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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
modificationsPhosphorylated by PKC. -
Cellular localization
Cytoplasm. - Information by UniProt
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Database links
- Entrez Gene: 10020 Human
- Entrez Gene: 50798 Mouse
- Entrez Gene: 114711 Rat
- Omim: 603824 Human
- SwissProt: Q9Y223 Human
- SwissProt: Q91WG8 Mouse
- SwissProt: O35826 Rat
- Unigene: 5920 Human
see all -
Alternative names
- 2310066H07Rik antibody
- Bifunctional UDP N acetylglucosamine 2 epimerase/N acetylmannosamine kinase antibody
- DMRV antibody
see all
Images
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PFA-fixed, Triton X-100 permeabilized A431 (human epidermoid carcinoma cell line) cells stained for GNE (green) using ab244403 at 4 µg/ml in ICC/IF.
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Paraffin-embedded human liver tissue stained for GNE using ab244403 at 1/50 dilution in immunohistochemical analysis.
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Anti-GNE antibody (ab244403) at 0.4 µg/ml + A549 (Human lung carcinoma cell line) cell lysate
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
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SDS download
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Datasheet download
References (0)
ab244403 has not yet been referenced specifically in any publications.