Azzo, A.

fibroblast enzyme activity deficiency s-galactosidase lysosomal neuraminidase disease i-cell lysosomal enzymes medium patient dalton factor study -/neur fusion control gangliosidosi precursor s-gal protein neuraminidase deficiency galjaard defect result neufeld neuraminidase activity hydrolase glycoprotein s-gal -/neur fibroblasts galactosidase biochem lysosomal enzyme deficiency experiment lysosome genet review lysosomal hydrolases s-gal -/neur cells strain sandhoff complementation i-cell disease table oligosaccharide s-galactosidase deficiency mucolipidosi addition component hexosaminidase control fibroblasts intracellular mutation biophy mannose uptake variant presence correction processing sialidosi chern storage nature publication cell strains hoogeveen mannosidosi hasilik culture recognition s-galactosidase activity restoration substrate press hybridization culture medium polypeptide gm 1 gangliosidosis