Anti-Neu1 Antibody | MyBioSource MBS8245875 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Anti-Neu1 Antibody

catalog :

MBS8245875

quantity :

0.05 mL

price :

175 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human, mouse, rat

application :

western blot

more info or order :

MyBioSource product webpage

image

image 1 :

Western blot analysis of Neu1 expression in mouse liver (A), rat liver (B) whole cell lysates.

product information

catalog number :

MBS8245875

products type :

Antibody

products full name :

Anti-Neu1 Antibody

products short name :

[Neu1]

products name syn :

[NANH; Sialidase-1; Acetylneuraminyl hydrolase; G9 sialidase; Lysosomal sialidase; N-acetyl-alpha-neuraminidase 1]

other names :

[sialidase-1; Sialidase-1; sialidase-1; neuraminidase 1 (lysosomal sialidase); Acetylneuraminyl hydrolase; G9 sialidase; Lysosomal sialidase; N-acetyl-alpha-neuraminidase 1]

products gene name :

[NEU1]

other gene names :

[NEU1; NEU1; NEU; NANH; SIAL1; NANH]

uniprot entry name :

NEUR1_HUMAN

clonality :

Polyclonal

host :

Rabbit

reactivity :

Human, Mouse, Rat

sequence length :

415

specificity :

Recognizes endogenous levels of Neu1 protein.

purity :

The antibody was purified by immunogen affinity chromatography.

form :

Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.

storage stability :

Shipped at 4°C. Upon delivery aliquot and store at -20°C for one year. Avoid freeze/thaw cycles.

tested application :

Western Blot (WB)

app notes :

WB: 1/500 - 1/2000

image1 heading :

Western Blot (WB)

other info1 :

Immunogen: Recombinant full length protein of human Neu1

products description :

Rabbit polyclonal antibody to Neu1

ncbi gi num :

4557791

ncbi acc num :

NP_000425.1

ncbi gb acc num :

NM_000434.3

uniprot acc num :

Q99519

ncbi pathways :

Asparagine N-linked Glycosylation Pathway (1268714); Biosynthesis Of The N-glycan Precursor (dolichol Lipid-linked Oligosaccharide, LLO) And Transfer To A Nascent Protein Pathway (1268715); Glycosphingolipid Metabolism Pathway (1270099); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Pathway (1269956); Metabolism Of Lipids And Lipoproteins Pathway (1270001); Metabolism Of Proteins Pathway (1268677); Other Glycan Degradation Pathway (82976); Other Glycan Degradation Pathway (346)

ncbi summary :

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 summary :

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: Lipid Metabolism - sphingolipid; Motility/polarity/chemotaxis; EC 3.2.1.18; Glycan Metabolism - other glycan degradation; Hydrolase. Chromosomal Location of Human Ortholog: 6p21.3. Cellular Component: cell junction; cell surface; cytoplasmic membrane-bound vesicle; intracellular membrane-bound organelle; lysosomal lumen; lysosomal membrane; lysosome; membrane; plasma membrane. Molecular Function: exo-alpha-sialidase activity. Biological Process: ganglioside catabolic process; glycosphingolipid metabolic process; oligosaccharide catabolic process. Disease: Neuraminidase Deficiency

size1 :

0.05 mL

price1 :

175 USD

size2 :

0.1 mL

price2 :

220

size3 :

0.2 mL

price3 :

295

more info or order :

MyBioSource product webpage