GNE Recombinant Rabbit Monoclonal Antibody [JE64-81]
Catalog# HA721090
GNE Recombinant Rabbit Monoclonal Antibody [JE64-81]
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WB
-
IHC-P
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Human
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Mouse
-
Rat
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unconjugated
Overview
Product Name
GNE Recombinant Rabbit Monoclonal Antibody [JE64-81]
Antibody Type
Recombinant Rabbit monoclonal Antibody
Immunogen
Synthetic peptide within human GNE aa 673-722/722.
Species Reactivity
Human, Mouse, Rat
Validated Applications
WB, IHC-P
Molecular Weight
Predicted band size: 79 kDa
Positive Control
A549 cell lysate, HeLa cell lysate, Mouse liver tissue lysate, Rat liver tissue lysate, rat liver tissue.
Conjugation
unconjugated
Clone Number
JE64-81
RRID
Product Features
Form
Liquid
Concentration
Storage Instructions
Shipped at 4℃. Store at +4℃ short term (1-2 weeks). Store at -20℃ long term.
Storage Buffer
1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
Isotype
IgG
Purification Method
Protein A affinity purified.
Application Dilution
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WB
-
1:1,000
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IHC-P
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1:200
Target
Function
Bifunctional UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase is an enzyme that in humans is encoded by the GNE gene. The bifunctional enzyme, UDP-N-acetylglucosamine 2-epimerase regulates and initiates biosynthesis of N-acetylneuraminic acid (NeuAc), a precursor of sialic acids. UDP-GlcNAc 2-epimerase activity is rate-limiting for the biosynthesis of sialic acid and is required for sialylation in hematopoietic cells. The activity of the enzyme can be controlled at the transcriptional level and can affect the sialylation and function of specific cell surface molecules expressed on B cells and myeloid cells. Modification of cell surface molecules with sialic acid is crucial for their function in many biologic processes, including cell adhesion and signal transduction. Differential sialylation of cell surface molecules is also implicated in the tumorigenicity and metastatic behavior of malignant cells. Sialuria is a rare inborn error of metabolism characterized by cytoplasmic accumulation and increased urinary excretion of free NeuAc.
Background References
1. Pogoryelova O. et. al. GNE myopathy: from clinics and genetics to pathology and research strategies. Orphanet J Rare Dis. 2018 May
2. Carrillo N. et. al. GNE Myopathy: Etiology, Diagnosis, and Therapeutic Challenges. Neurotherapeutics. 2018 Oct
Subcellular Location
Cytoplasm, cytosol.
Synonyms
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
ExpandImages
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Western blot analysis of GNE on different lysates with Rabbit anti-GNE antibody (HA721090) at 1/1,000 dilution.
Lane 1: A549 cell lysate (20 µg/Lane)
Lane 2: HeLa cell lysate (20 µg/Lane)
Lane 3: Mouse liver tissue lysate (40 µg/Lane)
Lane 4: Rat liver tissue lysate (40 µg/Lane)
Predicted band size: 79 kDa
Observed band size: 71 kDa
Exposure time: 3 minutes; ECL: K1802;
4-20% SDS-PAGE gel.
Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (HA721090) at 1/1,000 dilution was used in primary antibody dilution (K1803) at 4℃ overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1/50,000 dilution was used for 1 hour at room temperature. -
Immunohistochemical analysis of paraffin-embedded rat liver tissue with Rabbit anti-GNE antibody (HA721090) at 1/200 dilution.
The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (HA721090) at 1/200 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"