Specifications
Species Reactivity
Human; Mouse; Rat
Immunogen
NEU1 (NP_000425, 334 a.a. ~ 415 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Target
Alternative Names
NEU1; sialidase 1 (lysosomal sialidase); NEU; sialidase-1; G9 sialidase; exo-alpha-sialidase; lysosomal sialidase; acetylneuraminyl hydrolase; N-acetyl-alpha-neuraminidase 1; NANH; SIAL1; FLJ93471;
Product Background
Antigen 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.
Pathway
Glycosphingolipid metabolism, organism-specific biosystem; Lysosome, organism-specific biosystem; Lysosome, conserved biosystem; Metabolism, organism-specific biosystem; Metabolism of lipids and lipoproteins, organism-specific biosystem; Other glycan degradation, organism-specific biosystem; Other glycan degradation, conserved biosystem;
Citations
Publication ()
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