Publications by authors named "Jos Hoekman"

There are currently seven different zebrafish strains that model Dravet Syndrome, a severe childhood form of epilepsy. These models are based on a set of duplicated genes, and , which are the homologs for human . Disrupting one of the genes would mimic a heterozygous disease state in humans, as the paralog gene is still present.

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Dravet syndrome (DS) is a monogenic epileptic encephalopathy caused by loss-of-function mutations in the voltage-gated sodium channel (VGSC) gene . DS has an age of onset within the first year of life and severe disease prognosis. In the past years, it has been shown that upregulation of endogenous can be beneficial in animal models for DS, but a complete rescue was not observed.

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