Comparison of the alpha-mannosidases in fibroblast cultures from patients with mannosidosis and mucolipidosis II and from controls.
The intracellular and extracellular acidic alpha-mannosidase in cultures of fibroblasts from mucolipidosis-II patients has normal kinetics. The extracellular activity in cultures of cells from mannosidosis patients is normal, but a mutant enzyme is associated with the cell surface and intracellular fraction. The results support the involvement of membrane cycling and recognition markers in lysosomal enzyme localization.
D002478:Cells, Cultured; D003035:Cobalt / Q000494:pharmacology; D005347:Fibroblasts / Q000201:enzymology; D006801:Humans; D007424:Intracellular Fluid / Q000378:metabolism; D007700:Kinetics; D008361:Mannosidases / Q000172:deficiency / Q000378:metabolism*; D009081:Mucolipidoses / Q000201:enzymology*; D015032:Zinc / Q000494:pharmacology
D003035:Cobalt; D008361:Mannosidases; D015032:Zinc