This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Recent magnetic resonance imaging (MRI) studies have indicated that white matter abnormalities are an early hallmark of Sturge-Weber Syndrome (SWS) and are strongly correlated with cognitive decline. These white matter changes have been linked to acclerated myelination. Identifying the mechanisms underlying hypermyelination in SWS could therefore be a significant step towards finding ways to delay or inhibit cognitive decline and possibly other cortical abnormalities in affected patients. Our hypothesis is that gliotic astrocytes in SWS, either by themselves or in response to signals from vascular endothelial cells of leptomeningial angiomas, produce elevated VEGF (or related factors) that causes accelerated OPC proliferation and subsequent hypermyelination. We will test this hypothesis using a combination of patient-derived primary cells and murine cell culture models of oligodendrocyte progenitors.
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