The natural substrate, galactosylceramide (galactocerebroside), is used in this assay. The galactose moiety of the substrate is labeled with tritium. The liberated (3)H galactose is determined after it is separated from the unreacted galactosylceramide by solvent partitioning.(Suzuki K: Enzymic diagnosis of sphingolipidoses: globoid cell leukodystrophy [Krabbe's disease]. Methods Enzymol 1979;50:456-488; Radin NS: Labeled galactosyl ceramide and lactosyl ceramide. Methods Enzymol 1972;28:300-306)
Specimens are processed Monday through Sunday. Assay is set-up Wednesday, reported Thursday; 8 a.m.
WBC homogenate stored 1 month