Abstract
Lysosomal neuraminidase (NEU1) is the glycosidase responsible for the catabolism of sialic acid-containing glycoconjugates. A deficiency of this enzyme is associated with the lysosomal storage disease sialidosis. Children affected by this disease have severe systemic and neurological abnormalities, which include muscle hypotonia, atrophy and osteoskeletal deformities. Neu1-/- mice are a c…