Rabbit anti-Human NEU1 Polyclonal Antibody | anti-NEU1 antibody

NEU1, ID (NEU1, NANH, Sialidase-1, Acetylneuraminyl hydrolase, G9 sialidase, Lysosomal sialidase, N-acetyl-alpha-neuraminidase 1) (PE)

Cat. Num. AAA6324750

• Gene Names: NEU1; NEU; NANH; SIAL1
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: NEU1; Polyclonal Antibody; ID (NEU1; NANH; Sialidase-1; Acetylneuraminyl hydrolase; G9 sialidase; Lysosomal sialidase; N-acetyl-alpha-neuraminidase 1) (PE); N-acetyl-alpha-neuraminidase 1; anti-NEU1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with R-Phycoerythrin (PE).

• Applicable Applications for anti-NEU1 antibody: Western Blot (WB), FLISA
• Application Notes: WB: 1:100-500; FLISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: NEU1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 188-214 amino acids from the Central region of human NEU1.
• Conjugate: PE
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: PE conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(NEU1 Antibody (Center) western blot analysis in HepG2 cell line lysates (35ug/lane).This demonstrates the NEU1 antibody detected the NEU1 protein (arrow).)

Related Product Information for anti-NEU1 antibody

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.

Product Categories/Family for anti-NEU1 antibody

Antibodies; Enzymes; Glycosidase

References

Caciotti, A., et al. J. Neurol. 256(11):1911-1915(2009). Bonten, E.J., et al. J. Biol. Chem. 284(41):28430-28441(2009). Barcellos, L.F., et al. PLoS Genet. 5 (10), E1000696 (2009): Wang, J., et al. J. Neurochem. 111(2):547-554(2009). Lai, S.C., et al. Eur. J. Neurol. 16(8):912-919(2009).

NCBI and Uniprot Product Information

• NCBI GI #: 4557791
• NCBI GeneID: 4758
• NCBI Accession #: NP_000425.1
• NCBI GenBank Nucleotide #: NM_000434.3
• UniProt Accession #: Q99519
• NCBI Official Full Name: sialidase-1
• NCBI Official Synonym Full Names: sialidase 1 (lysosomal sialidase)
• NCBI Official Symbol: NEU1
• NCBI Official Synonym Symbols: NEU; NANH; SIAL1
• NCBI Protein Information: sialidase-1; G9 sialidase; N-acetyl-alpha-neuraminidase 1; acetylneuraminyl hydrolase; exo-alpha-sialidase; lysosomal sialidase
• UniProt Protein Name: Sialidase-1
• Protein Family: Sialidase
• UniProt Gene Name: NEU1
• UniProt Synonym Gene Names: NANH
• UniProt Entry Name: NEUR1_HUMAN

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