Glycosphingolipid Expression in Cerebrospinal Fluid of Infants with Neurological Abnormalities: Report of Three CasesReport as inadecuate




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Collegium antropologicum, Vol.32 - Supplement 1 No.1 May 2008. -

The aim of this study was to analyse glycosphingolipid expression in cerebrospinal fluid CSF from one idiopathic

West syndrome IWS infant, one with Reye like syndrome, and one with congenital hydrocephalus, in comparison to

control group n=7 using highly sensitive thin-layer chromatography-immunostaining methods. Gangliotetraose-series

gangliosides acidic glycosphingolipids were not detected in CSF of infant with idiopathic West syndrome and infant

with congenital hydrocephalus. CSF of infant with IWS showed traces of neolacto-tetraose ganglioside fractions,

which were absent in all other CSF examined. In addition, lactosylceramide fraction, and one ceramide fraction were

highly expressed only in IWS CSF. These results confirmed previously described lack of gangliotetraose-series gangliosides

in IWS patient and for the first time is described increased expression of neolacto-series glycosphingolipids in IWS

patient. Since follow up until the age of five years showed almost normal IWS patient psychomotor development, the

discribed shift of glycosphingolipid expression may implicate on transient inhibition of specific glycosyl transferases in

the age of seven months.

idiopathic West syndrome; Reye like syndrome; congenital hydrocephalus; cerebrospinal fluid; glycosphingolipids



Author: Maja Tomasović -

Source: http://hrcak.srce.hr/



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