Ostalo
Glycosphingolipid Expression in Cerebrospinal Fluid of Infants with Neurological Abnormalities: Report of Three Cases
Maja Tomasović
Sažetak
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.
Ključne riječi
idiopathic West syndrome; Reye like syndrome; congenital hydrocephalus; cerebrospinal fluid; glycosphingolipids
Hrčak ID:
24463
URI
Datum izdavanja:
13.5.2008.
Posjeta: 866 *