Lysosomal glycosidase activities in human hair roots.
The levels of three lysosomal glycosidase, alpha-D-mannosidase, a-L-fucosidase and beta-D-hexosaminidase have been determined in normal hair roots and in hair roots obtained from a patient with mannosidosis. The most active glycosidase in normal hair roots was beta-D-hexosaminidase, followed by alpha-L-fucosidase and alpha-D-mannosidase. There was no alpha-D-mannosidase activity in the hair roots of the patient with mannosidosis. The significance of these results is discussed in relation to the detection of lysosomal storage diseases.
D002239:Carbohydrate Metabolism, Inborn Errors / Q000175:diagnosis; D006026:Glycoside Hydrolases / Q000378:metabolism*; D006197:Hair / Q000166:cytology*; D006596:Hexosaminidases / Q000378:metabolism; D006801:Humans; D006863:Hydrogen-Ion Concentration; D008247:Lysosomes / Q000201:enzymology*; D008358:Mannose / Q000378:metabolism; D008361:Mannosidases / Q000172:deficiency / Q000378:metabolism; D008722:Methods; D005644:alpha-L-Fucosidase / Q000378:metabolism
D006026:Glycoside Hydrolases; D006596:Hexosaminidases; D008361:Mannosidases; D005644:alpha-L-Fucosidase; D008358:Mannose