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The <jats:italic>Cyfip2<\/jats:italic><jats:sup><jats:italic>+\/R87C<\/jats:italic><\/jats:sup> mice recapitulated many neurological and neurobehavioral phenotypes of the patients, including spasmlike movements, microcephaly, and impaired social communication. Age\u2010progressive cytoarchitectural disorganization and gliosis were also identified in the hippocampus of <jats:italic>Cyfip2<\/jats:italic><jats:sup><jats:italic>+\/R87C<\/jats:italic><\/jats:sup> mice. Beyond identifying a decrease in CYFIP2 protein levels in the <jats:italic>Cyfip2<\/jats:italic><jats:sup><jats:italic>+\/R87C<\/jats:italic><\/jats:sup> brains, we demonstrated that the p.Arg87Cys variant enhances ubiquitination and proteasomal degradation of CYFIP2. 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