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Maternal Loss of Ube3a Impairs Experience-Driven Dendritic Spine Maintenance in the Developing Visual Cortex.

Hyojin KimPortia A KunzRichard MooneyBenjamin D PhilpotSpencer LaVere Smith
Published in: The Journal of neuroscience : the official journal of the Society for Neuroscience (2017)
Reduced dendritic spine densities are common in the neurodevelopmental disorder Angelman syndrome (AS). Because prior reports were based on postmortem tissue, it was unknown whether this anatomical deficit arises from decreased spine formation and/or increased spine elimination. Here, we used in vivo two-photon imaging to track spines over multiple days in a mouse model of AS. We found that spine formation is normal, but experience-dependent spine maintenance is reduced in the visual cortex of AS model mice. Our data pinpoint the anatomical process underlying the loss of dendritic spines, which can account for the decreased excitatory synaptic connectivity associated with AS. Therefore, normalizing spine maintenance is a potential therapeutic strategy.
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