Rescue of neurological deficits in a mouse model for Angelman syndrome by reduction of αCaMKII inhibitory phosphorylation

Geeske M. Van Woerden, Karen D. Harris, Mohammad Reza Hojjati, Richard M. Gustin, Shenfeng Qiu, Rogerio De Avila Freire, Yong Hui Jiang, Ype Elgersma, Edwin J. Weeber

Research output: Contribution to journalArticle

195 Scopus citations

Abstract

Angelman syndrome (AS) is a severe neurological disorder characterized by mental retardation, motor dysfunction and epilepsy. We show that the molecular and cellular deficits of an AS mouse model can be rescued by introducing an additional mutation at the inhibitory phosphorylation site of αCaMKII. Moreover, these double mutants no longer show the behavioral deficits seen in AS mice, suggesting that these deficits are the direct result of increased inhibitory phosphorylation of αCaMKII.

Original languageEnglish (US)
Pages (from-to)280-282
Number of pages3
JournalNature neuroscience
Volume10
Issue number3
DOIs
StatePublished - Mar 1 2007
Externally publishedYes

ASJC Scopus subject areas

  • Neuroscience(all)

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    Van Woerden, G. M., Harris, K. D., Hojjati, M. R., Gustin, R. M., Qiu, S., De Avila Freire, R., Jiang, Y. H., Elgersma, Y., & Weeber, E. J. (2007). Rescue of neurological deficits in a mouse model for Angelman syndrome by reduction of αCaMKII inhibitory phosphorylation. Nature neuroscience, 10(3), 280-282. https://doi.org/10.1038/nn1845