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