A simple method of lysosomal hydrolase assay in a single somatic cell and its application.
A new and simple assay method for lysosomal hydrolase in a single cultured skin fibroblast is described. Using 4-methylumbelliferyl-glycosides and ordinary laboratory equipment, the activity can be measured with the highest sensitivity of 0.5 pmol of 4-methylumbelliferone released from the substrates. Two systems were compared and the methods described proved reliable for the diagnosis of several variants of beta-galactosidase deficiency.
D005347:Fibroblasts / Q000201:enzymology; D006026:Glycoside Hydrolases / Q000032:analysis*; D006801:Humans; D006923:Hymecromone / Q000378:metabolism; D008247:Lysosomes / Q000201:enzymology*; D008722:Methods
D006923:Hymecromone; D006026:Glycoside Hydrolases