Figure 5.

Psychosine accumulation disrupts raft architecture in brains of human Krabbe patients. A, Mass spectrometric analysis of human samples is expressed in picomoles per gram and reveals that psychosine preferentially accumulates in lipid rafts in humans with Krabbe disease. B, Fluorometric cholesterol assays of human samples are expressed as a percentage of the total and show that cholesterol accumulation accompanies the accumulation of psychosine. C, Western blotting was used to analyze fractions prepared from human tissue for distributions of the lipid raft-marker proteins Flotillin-2 (Flot 2) and Caveolin-1 (Cav-1). The results show a disruption of these proteins in a pattern similar to that observed in the TWI mouse. P115 was again used as a marker for nonrafts.