NEU1 encodes the lysosomal enzyme neuraminidase, Neu1, which cleaves terminal sialic acid residues from substrates such as glycoproteins and glycolipids .
In the lysosome Neu1 belongs to a heterotrimeric complex containing beta-galactosidase and cathepsin A (also referred to as 'protective protein') .
In humans, primary or secondary deficiency of this enzyme leads to two clinically similar neurodegenerative lysosomal storage disorders: sialidosis and galactosialidosis (GS) .
Sialidosis symptoms range from eye abnormalities and neurological disturbances to skeletal malformations, mental retardation and early death.
Neu1 is expressed in the pancreas, muscle, kidney, placenta, heart, lung and liver.
The human Neu1 gene maps to chromosome 6p21.33 and encodes a lysosomal protein localized on the inner side of the plasma membrane and in intracellular vesicles .
Neu1 is also known as alpha-N-acetyl-neuraminidase and Acetylneuraminyl hydrolase .
Applications:Suitable for use in Western Blot, Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：50ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid PBS, 0.1% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of NEU1. Species Crossreactivity: Human
