Seizure-like activity in a juvenile Angelman syndrome mouse model is attenuated by reducing Arc expression
Abstract
Angelman syndrome (AS) is a human neurodevelopmental disorder caused by mutation of a specific gene, UBE3A. Studies of behavior in adult mouse models of AS reveal abnormalities similar to those observed in humans with AS. Because AS affects children, we hypothesized that it might be helpful to study this disorder using juvenile mice. We found that young AS mice display aberrant communication and motor behaviors and increased brain activity. Reducing the expression of the synaptic protein ARC reverses abnormal brain activity in AS mice, but has no effect on communication and motor behaviors in these mice. These findings suggest new approaches for identifying the neural circuits that are defective in AS, and for developing therapies for treating this disorder.
- Publication:
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Proceedings of the National Academy of Science
- Pub Date:
- April 2015
- DOI:
- 10.1073/pnas.1504809112
- Bibcode:
- 2015PNAS..112.5129M