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NEU1/Neuraminidase Rabbit pAb

NEU1/Neuraminidase Rabbit pAb

     
  • 1 - NEU1/Neuraminidase Rabbit pAb AP59051
    Sample:U251 (human)cell Lysate at 40 ug
    Primary: Anti-NEU1/Neuraminidase(AP59051)at 1/300 dilution
    Secondary: IRDye800CW Goat Anti-RabbitIgG at 1/20000 dilution
    Predicted band size: 45kD
    Observed band size: 45kD
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB
Primary Accession Q99519
Reactivity Human
Predicted Rat
Host Rabbit
Clonality Polyclonal
Calculated MW 45467 Da
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human NEU1/Neuraminidase
Epitope Specificity 151-250/415
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SUBCELLULAR LOCATION Lysosome membrane. Lysosome lumen. Cell membrane. Cytoplasmic vesicle. Localized not only on the inner side of the lysosomal membrane and in the lysosomal lumen, but also on the plasma membrane and in intracellular vesicles.
DISEASE Defects in NEU1 are the cause of sialidosis (SIALIDOSIS) [MIM:256550]. It is a lysosomal storage disease occurring as two types with various manifestations. Type 1 sialidosis (cherry red spot-myoclonus syndrome or normosomatic type) is late-onset and it is characterized by the formation of cherry red macular spots in childhood, progressive debilitating myoclonus, insiduous visual loss and rarely ataxia. The diagnosis can be confirmed by the screening of the urine for sialyloligosaccharides. Type 2 sialidosis (also known as dysmorphic type) occurs as several variants of increasing severity with earlier age of onset. It is characterized by the presence of abnormal somatic features including coarse facies and dysostosis multiplex, vertebral deformities, mental retardation, cherry-red spot/myoclonus, sialuria, cytoplasmic vacuolation of peripheral lymphocytes, bone marrow cells and conjunctival epithelial cells.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions The protein encoded by this gene is a lysosomal enzyme that cleaves terminal sialic acid residues from substrates such as glycoproteins and glycolipids. In the lysosome, this enzyme is part of a heterotrimeric complex together with beta-galactosidase and cathepsin A (the latter is also referred to as 'protective protein'). Mutations in this gene can lead to sialidosis, a lysosomal storage disease that can be type 1 (cherry red spot-myoclonus syndrome or normosomatic type), which is late-onset, or type 2 (the dysmorphic type), which occurs at an earlier age with increased severity. [provided by RefSeq, Jul 2008]
Additional Information
Gene ID 4758
Other Names Sialidase-1, 3.2.1.18, Acetylneuraminyl hydrolase, G9 sialidase, Lysosomal sialidase, N-acetyl-alpha-neuraminidase 1, NEU1, NANH
Dilution WB=1:500-2000
StorageStore at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name NEU1 (HGNC:7758)
Synonyms NANH
Function Lysosomal exo-alpha-sialidase that catalyzes the removal of sialic acid (N-acetylneuraminic acid) moieties from glycoproteins and glycolipids. To be active, it is strictly dependent on its presence in the multienzyme complex (PubMed:14695530, PubMed:25153125, PubMed:37205763, PubMed:8985184, PubMed:9054950). Appears to have a preference for alpha 2-3 and alpha 2-6 sialyl linkage.
Cellular Location Lysosome lumen. Lysosome membrane; Peripheral membrane protein; Lumenal side. Cell membrane; Peripheral membrane protein. Note=Localized not only on the inner side of the lysosomal membrane and in the lysosomal lumen, but also on the plasma membrane and in intracellular vesicles (PubMed:11571282, PubMed:9480870). Was also suggested to have transmembrane topology (PubMed:27917893).
Tissue Location Highly expressed in pancreas, followed by skeletal muscle, kidney, placenta, heart, lung and liver. Weakly expressed in brain.
Research Areas

BACKGROUND

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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