MECHANISMS OF OLIGODENDROCYTE DYSFUNCTION IN MILD MALFORMATION OF CORTICAL DEVELOPMENT WITH OLIGODENDROGLIAL HYPERPLASIA AND EPILEPSY
Public support
Provider
Czech Science Foundation
Programme
Standard projects
Call for proposals
SGA0202600001
Main participants
Univerzita Karlova / 2. lékařská fakulta
Contest type
VS - Public tender
Contract ID
26-22519S
Alternative language
Project name in Czech
MECHANISMS OF OLIGODENDROCYTE DYSFUNCTION IN MILD MALFORMATION OF CORTICAL DEVELOPMENT WITH OLIGODENDROGLIAL HYPERPLASIA AND EPILEPSY
Annotation in Czech
Drug-resistant epilepsy in pediatric populations remains a significant challenge, with mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE) identified as one of the causes. MOGHE is characterized by heterotopic neurons, oligodendrocyte hyperplasia, and patchy hypomyelination, often linked to somatic variants in the SLC35A2 gene. This project aims to investigate the cellular and molecular mechanisms underlying MOGHE, with a focus on oligodendrocyte lineage cells. We hypothesize that disrupted glycosylation, resulting from SLC35A2 mutations, impairs the differentiation of oligodendrocyte precursor cells, leading to hypomyelination, potentially contributing to epileptogenesis. The study will investigate the roles of NMDA and GPR17 receptors in this process, glycosylation deficits in oligodendrocyte lineage cells and myelin, and assess the feasibility of D-galactose supplementation therapy using in silico analysis of the mutated protein structure, with the aim of uncovering potential therapeutic targets for patients with MOGHE.
Scientific branches
R&D category
ZV - Basic research
OECD FORD - main branch
30103 - Neurosciences (including psychophysiology)
OECD FORD - secondary branch
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OECD FORD - another secondary branch
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CEP - equivalent branches <br>(according to the <a href="http://www.vyzkum.cz/storage/att/E6EF7938F0E854BAE520AC119FB22E8D/Prevodnik_oboru_Frascati.pdf">converter</a>)
FH - Neurology, neuro-surgery, nuero-sciences
Solution timeline
Realization period - beginning
Jan 1, 2026
Realization period - end
Dec 31, 2028
Project status
Z - Beginning multi-year project
Latest support payment
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Data delivery to CEP
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data delivery code
CEP26-GA0-GA-R
Data delivery date
May 19, 2026
Finance
Total approved costs
9,792 thou. CZK
Public financial support
9,792 thou. CZK
Other public sources
0 thou. CZK
Non public and foreign sources
0 thou. CZK