Abstract
The neuraminidase-1 (Neu1) regulates the catabolism of sialoglycoconjugates in lysosomes. Congenital deficiency of Neu1 is the basis of sialidosis, a severe neurosomatic disease associated with osteoskeletal deformities, hypotonia and muscle weakness. Neu1 deficient mice (Neu1-/-) develop an atypical form of muscular degeneration characterized by expansion of extracellular matrix (ECM), i…