|Other Accession||A6BMK7, NP_000425.1|
|Other Names||Sialidase-1, Acetylneuraminyl hydrolase, G9 sialidase, Lysosomal sialidase, N-acetyl-alpha-neuraminidase 1, NEU1, NANH|
|Target/Specificity||The synthetic peptide sequence is selected from aa 203-214 of HUMAN NEU1|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Catalyzes the removal of sialic acid (N-acetylneuraminic 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.|
|Cellular Location||Lysosome membrane; Peripheral membrane protein; Lumenal side. Lysosome lumen. Cell membrane. Cytoplasmic vesicle. Lysosome. 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|
|Tissue Location||Highly expressed in pancreas, followed by skeletal muscle, kidney, placenta, heart, lung and liver. Weakly expressed in brain.|
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Provided below are standard protocols that you may find useful for product applications.
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.
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)
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