A comprehensive functional analysis of Dup15q ‎genes: phenotypic effects and treatment ‎following restoration‎

Awardee: Alon Zaslaver

Institution: The Hebrew University

Grant Amount: $47,038

Funding Period: February 1, 2025 - January 31, 2026


Summary:

Dup15q syndrome is genetically inherited and is caused due to an extra copy of a piece of ‎chromosome ‎‎15. As a results, the genes located on that chromosome region are expressed at ‎higher levels. However, ‎it is less clear which genes contribute to the various observed deficits. ‎Moreover, unless genetically tested during pregnancy, children with Dup15q are typically ‎‎diagnosed only at the age of ~2 years old. Thus, new therapeutic approaches need to focus on ‎‎improving and restoring functional deficits after most embryonic neurodevelopmental processes ‎are complete.‎ To address all these needs, we will use the powerful genetic model system (C. elegans worms). ‎Specifically, we ‎carefully designed an experimental genetic system which allows mild and fine ‎upregulation of individual and combinations of Dup15q genes during neurodevelopment, and ‎restoration of the elevated expression to normal ‎levels at the adult stage of the animal. To ‎analyze neurodevelopmental deficits, we will ‎use state-of-the-art experimental techniques ‎including functional imaging of neural activity and behavioral ‎assays.‎ These efforts will reveal the individual and sets of genes that lead to neurodevelopmental ‎phenotypes, and most importantly, whether restoration of gene expression to normal levels,‎ can improve these phenotypic deficits. Such findings may pave the way to novel interventions ‎and ‎therapeutic approaches.‎

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A novel RNA therapeutic for the treatment of CTNNB1 syndrome

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Molecular and Functional Mechanisms Underlying Cortical Activity in CACNA1A Epilepsy