The first sentence of a paragraph that begins on page 710 should have read, "These results suggest that measuring circulating blood levels of psychosine in patients with KD may serve as endpoints for correlation with their neurologic phenotype.
Detection of the neurotoxin psychosine in samples of peripheral blood: application in diagnostics and follow-up of krabbe disease.
GALC is responsible for the lysosomal hydrolysis of certain galactolipids located mainly in myelin, galactosylceramide and psychosine
Sphingosylphosphoryl choline (~85%), psychosine, Triton X-100, sodium taurocholate, and oleic acid were purchased from Sigma.
The desired compounds were made using the same procedure to make compounds 7 and 8 except that sphingosine was used in place of sphingosylphosphoryl choline and psychosine, respectively.
The toxic fat psychosine also accumulates in the central nervous system, and physical symptoms similar to those of Krabbe's disease develop in affected mice, which die about five weeks after birth.
Results also show that marrow transplants -- performed when the mice are 9 to 12 days old -- increase enzyme levels in the central nervous system, result in much lower levels of psychosine and are followed by a disappearance of globoid cells.
Predictably, affected individuals show progressive accumulation of psychosine in central and peripheral nerve tissue.
We examined whether psychosine was present in detectable quantities in the blood of the twitcher mouse, a natural occurring murine model of KD.
Dried blood spots were then maintained frozen for about a week before psychosine quantification.