Immunofluorescence (IF)
Rabbit NEU1 Polyclonal Antibody | anti-NEU1 antibody
Anti NEU1 polyclonal antibody
Other species were not tested
Other species were not tested
IHC: 1:200-1:400
Immunofluorescence (IF)
Immunofluorescence (IF)
Western Blot (WB)
Western Blot (WB)
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
NEU1: Catalyzes the removal of sialic acid (N-acetylneuramic acid) moities from glycoproteins and glycolipids. To be active, it is strictly dependent on its presence in the multienzyme complex. Appears to have a preference for alpha 2-3 and alpha 2-6 sialyl linkage. Defects in NEU1 are the cause of sialidosis (SIALIDOSIS). 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. Belongs to the glycosyl hydrolase 33 family.
Protein type: EC 3.2.1.18; Motility/polarity/chemotaxis; Glycan Metabolism - other glycan degradation; Hydrolase; Lipid Metabolism - sphingolipid
Chromosomal Location of Human Ortholog: 6p21.3
Cellular Component: lysosomal lumen; intracellular membrane-bound organelle; lysosome; lysosomal membrane; cytoplasmic membrane-bound vesicle; plasma membrane; cell junction
Molecular Function: exo-alpha-sialidase activity
Biological Process: oligosaccharide catabolic process; cellular protein metabolic process; sphingolipid metabolic process; dolichol-linked oligosaccharide biosynthetic process; protein amino acid N-linked glycosylation via asparagine; glycosphingolipid metabolic process; post-translational protein modification; lipid catabolic process
Disease: Neuraminidase Deficiency
Research Articles on NEU1
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Product Notes
The NEU1 neu1 (Catalog #AAA1560322) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Anti NEU1 polyclonal antibody reacts with Human Other species were not tested and may cross-react with other species as described in the data sheet. AAA Biotech's NEU1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunohistochemistry (IHC). WB: 1:2000-1:8000 IHC: 1:200-1:400. Researchers should empirically determine the suitability of the NEU1 neu1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "NEU1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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