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

doi:10.1038/nn1845

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 journal › Article

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 language	English (US)
Pages (from-to)	280-282
Number of pages	3
Journal	Nature neuroscience
Volume	10
Issue number	3
DOIs	https://doi.org/10.1038/nn1845
State	Published - Mar 1 2007
Externally published	Yes

ASJC Scopus subject areas

Neuroscience(all)

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10.1038/nn1845

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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

Rescue of neurological deficits in a mouse model for Angelman syndrome by reduction of αCaMKII inhibitory phosphorylation. / Van Woerden, Geeske M.; Harris, Karen D.; Hojjati, Mohammad Reza; Gustin, Richard M.; Qiu, Shenfeng; De Avila Freire, Rogerio; Jiang, Yong Hui; Elgersma, Ype; Weeber, Edwin J.

In: Nature neuroscience, Vol. 10, No. 3, 01.03.2007, p. 280-282.

Research output: Contribution to journal › Article

Van Woerden, Geeske M. ; Harris, Karen D. ; Hojjati, Mohammad Reza ; Gustin, Richard M. ; Qiu, Shenfeng ; De Avila Freire, Rogerio ; Jiang, Yong Hui ; Elgersma, Ype ; Weeber, Edwin J. / Rescue of neurological deficits in a mouse model for Angelman syndrome by reduction of αCaMKII inhibitory phosphorylation. In: Nature neuroscience. 2007 ; Vol. 10, No. 3. pp. 280-282.

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