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 › peer-review

208 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 2007
Externally published	Yes

ASJC Scopus subject areas

Neuroscience(all)

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10.1038/nn1845

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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, 03.2007, p. 280-282.

Research output: Contribution to journal › Article › peer-review

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

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.",

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

note = "Funding Information: The authors thank M. Elgersma and M. Aghadavoud Jolfaei for technical support. This work was supported by the Netherlands Organization for Scientific Research (NWO-ZonMW), Hersenstichting Nederland (HsN), Netherlands Epilepsy Fund and the Nina Foundation/Angelman Vereniging.",

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doi = "10.1038/nn1845",

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AU - Van Woerden, Geeske M.

AU - Harris, Karen D.

AU - Hojjati, Mohammad Reza

AU - Gustin, Richard M.

AU - Qiu, Shenfeng

AU - De Avila Freire, Rogerio

AU - Jiang, Yong Hui

AU - Elgersma, Ype

AU - Weeber, Edwin J.

N1 - Funding Information: The authors thank M. Elgersma and M. Aghadavoud Jolfaei for technical support. This work was supported by the Netherlands Organization for Scientific Research (NWO-ZonMW), Hersenstichting Nederland (HsN), Netherlands Epilepsy Fund and the Nina Foundation/Angelman Vereniging.

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AB - 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.

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