Endocytosis of beta-N-acetylglucosaminidase from sections of mucolipidosis-II and-III fibroblasts by non-parenchymal rat liver cells.
beta-N-Acetylglucosaminidase isolated from the secretions of fibroblasts of mucolipidosis-II and -III patients is internalized by cultured non-parenchymal rat liver cells. The rate of endocytosis compared with that of beta-N-acetylglucosaminidase from control fibroblasts was 11 and 19% for the enzyme from mucolipidosis-II and -III patients respectively. The inhibition of endocytosis by mannan indicates that the beta-N-acetylglucosaminidase from mucolipidosis-II and -III patients is recognized by cell-surface receptors specific for mannose.
D000118:Acetylglucosaminidase / Q000378:metabolism*; D000818:Animals; D004705:Endocytosis; D005347:Fibroblasts / Q000201:enzymology / Q000378:metabolism; D006596:Hexosaminidases / Q000378:metabolism*; D006801:Humans; D066298:In Vitro Techniques; D008099:Liver / Q000166:cytology / Q000378:metabolism*; D009081:Mucolipidoses / Q000201:enzymology*; D051381:Rats
D006596:Hexosaminidases; D000118:Acetylglucosaminidase