The assay of sphingolipid hydrolases in white blood cells with labelled natural substrates.
Methods were elaborated for the assay on leukocytes of sphingolipid hydrolases with native and artificial substrates. The optimal conditions for the isolation of leukocytes and the preparation of labelled sphingolipids were investigated. The KM and Vmax values for all substrates are given as the specific activities of the sphingolipidhydrolases in normal males and females. It is concluded that leukocytes are the enzyme source of choice for the enzymic diagnosis of neurolipidoses.
D000328:Adult; D003902:Detergents / Q000494:pharmacology; D005260:Female; D005732:Gangliosides; D006026:Glycoside Hydrolases / Q000097:blood*; D006028:Glycosphingolipids; D006801:Humans; D006867:Hydrolases / Q000097:blood*; D066298:In Vitro Techniques; D007700:Kinetics; D007962:Leukocytes / Q000201:enzymology*; D008297:Male; D008875:Middle Aged; D012995:Solubility; D013107:Sphingolipids; D013109:Sphingomyelins
D003902:Detergents; D005732:Gangliosides; D006028:Glycosphingolipids; D013107:Sphingolipids; D013109:Sphingomyelins; D006867:Hydrolases; D006026:Glycoside Hydrolases