To explore the mechanisms underlying the neurological symptoms of IP, we investigated mice with a germline deletion or with cell type–specific deletions of Nemo in the CNS. Deletion of Nemo in brain endothelial cells resulted in disruption of the BBB and endothelial cell dysfunction and death. Our data dissect the pathways that disturb brain endothelial function and lead to the neurological manifestations of IP when NEMO is inactivated.

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