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Unit Code 8816:
Galactosylceramide Beta-Galactosidase, Leukocytes

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Method Description

The natural substrate, galactosylceramide (galactocerebroside), is

used in this assay.  The galactose moiety of the substrate has been

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)

Performing Laboratory Location

Rochester

Key