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Ube3a

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Study links chronic cellular stress response to Angelman syndrome symptoms in mice

Researchers at Washington University in St. Louis used Ube3a-deficient mice, a standard model of Angelman syndrome, along with a newly engineered Asns-mutant mouse line, to study neuronal stress signaling. They report that sustained activation of a cellular stress pathway in neurons contributes to the behavioral and neurological phenotypes seen in the Angelman mouse model.