Sialidase-1


Sialidase-1, is a mammalian lysosomal neuraminidase enzyme which in humans is encoded by the NEU1 gene.

Function

The protein SIALIDASE-1 encoded by the NEU-1 gene encodes the lysosomal enzyme SIALIDASE-1, which 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. Mutations in this gene can lead to sialidosis.

Clinical significance

Mutations in NEU1 leads to sialidosis, a rare lysosomal [storage disease]. Sialidase has also been shown to enhance recovery from spinal cord contusion injury when injected in rats.

Interactions

NEU1 has been shown to interact with Cathepsin A.