A potential source of error in the enzymatic diagnosis of the neurolipidoses when radiolabelled sphinogolipids are used as substrates.
High specific activity 3H-labelled glucocerebroside, galactocerebroside and sphingomyelin but not 3H-labelled ceramide, have been found to bind to the wall of glass scintillation vials thereby greatly decreasing the counting efficiency. The binding of the lipids was largely eliminated by counting samples in scintillation fluid containing methanol (6-10%, v/v). To avoid possible errors in the enzymatic diagnosis of the neurolipidoses, it is suggested that the latter solvent should be routinely incorporated into scintillation fluid used for counting labelled sphingolipids.
D002518:Ceramides; D006028:Glycosphingolipids; D006801:Humans; D006867:Hydrolases / Q000097:blood; D007553:Isotope Labeling; D008052:Lipid Metabolism, Inborn Errors / Q000175:diagnosis* / Q000201:enzymology; D012588:Scintillation Counting; D013109:Sphingomyelins; D014316:Tritium
D002518:Ceramides; D006028:Glycosphingolipids; D013109:Sphingomyelins; D014316:Tritium; D006867:Hydrolases