Naslov
Glycosphingolipid expression in cerebrospinal fluid of infants with neurological abnormalities

Autori
Tomasović, Maja ; Rešić, Biserka ; Ivelja, Nada ; Kuzmanić-Šamija, Radenka ; Rešić, Jasminka ; Božić, Joško

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
Neurologia Croatica 57 (2008) / Hajnšek, Sanja - Zagreb : Klinički bolnički centar Zagreb, Medicinski fakultet Sveučilišta u Zagrebu, 2008

Skup
20th Annual Meeting of the European Academy of Childhood Disability "Early Diagnosis Implies Early Intervention"

Mjesto i datum
Zagreb, Hrvatska, 05.06.2008. - 07.06.2008

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

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

Sažetak
The aim of this study was to analyse glycosphingolipids expression in cerebrospinal fluid (CSF) of infants with neurological abnormalities. Neurochemical understanding of the cell surface events that accompany neurogenesis is important for determining the pathogenesis of age-dependent diseases. Because they are adundant in the outer surface of neuronal plasma membrane, GSLs may play an important role in these events. CSF samples obtained from infants with neurological abnormalities (n=3) and from control group (n=7)were analyzed separately for GSL expression by highly sensitive thin-layer chromatography-immunostaining methods. Of the 3 infants aged 1-12 months with neurological abnormalities, one was diagnosed with idiopathic West syndrome (IWS), one with Reye like syndrome, and one with congenital hydrocephalus. We analysed these samples in comparison with CSF samples obtained from 7 infants admitted to our department for lumbar puncture due to seizures. 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. Using HPTL-immunostaining chromatography, it was possible to detect CSF GSLs in as little as 10-30 microl of 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 fransferases in the age of seven months.

Izvorni jezik
Engleski

Znanstvena područja
Temeljne medicinske znanosti, Kliničke medicinske znanosti

Napomena
Sažetak je objavljen u časopisu citiranom u Neuroscience Citation Index i EMBASE/Excerpta Medica. Neurologia Croatica. 57 (2008) , Suppl. 1

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