Abstract
Neuraminidases are enzymes that participate in the regulation of the catabolism of sialoglicoconjugates in the lisosomes. A congenital deficiency of the sialidase Neu1 in children is based on the sialidosis, a neurosomatic severe disease associated with symptoms of hypotonia, muscle weakness and osteoskeletal deformities. Objective: To investigate the involvement of different forms of sia…