Psychosine is a highly cytotoxic lipid that accumulates in the nervous system in the absence of galactosylceramidase and has been associated with Krabbe disease.
Formation The enzyme sphingosine beta-galactosyltransferase converts sphingosine to psychosine:
In this reaction, a galactose sugar from uridine diphosphate galactose is added to sphingosine at its terminal hydroxy group. Uridine diphosphate (UDP) is the byproduct.
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