Rabbit anti-Human NEU1 Polyclonal Antibody, FITC conjugated

Uniprot Id

Q99519

Target Species

Human

Target Name

NEU1

Target Full Name

Sialidase-1

Target Function

Catalyzes the removal of sialic acid (N-acetylneuraminic acid) moieties 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.

Target Involvement

Sialidosis (SIALIDOSIS)

Target Subcellular 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.

Target Protein Families

Glycosyl hydrolase 33 family

Target Tissue Specificity

Highly expressed in pancreas, followed by skeletal muscle, kidney, placenta, heart, lung and liver. Weakly expressed in brain.

Target Synonyms

Acetylneuraminyl hydrolase; exo-alpha-sialidase; G9 sialidase; Lysosomal sialidase; N acetyl alpha neuraminidase 1; N-acetyl-alpha-neuraminidase 1; NANH; NEU; NEU1; NEUR1_HUMAN; Neuraminidase 1; Neuraminidase; SIAL1; sialidase 1 (lysosomal sialidase); Sialidase 1; Sialidase; lysosomal; Sialidase-1

Target Background

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.