Brief Communication abstract
Nature Neuroscience 10, 280 - 282 (2007)
Published online: 28 January 2007 | Corrected online: 6 February 2007 | doi:10.1038/nn1845
Rescue of neurological deficits in a mouse model for Angelman syndrome by reduction of 
CaMKII inhibitory phosphorylation
Geeske M van Woerden1, Karen D Harris2, Mohammad Reza Hojjati1, Richard M Gustin3, Shenfeng Qiu2, Rogerio de Avila Freire1, Yong-hui Jiang4, Ype Elgersma1 & Edwin J Weeber2,3
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 
CaMKII. 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 CaMKII.
- Erasmus MC, University Medical Centre, Department of Neuroscience, Dr. Molewaterplein 50, PO Box 2040, 3000 CA, Rotterdam, The Netherlands.
- Department of Molecular Physiology and Biophysics, Vanderbilt University, 754 Robinson Research Building, Nashville, Tennessee 37235, USA.
- Department of Pharmacology, Vanderbilt University, 754 Robinson Research Building, Nashville, Tennessee 37235, USA.
- Department of Molecular and Human Genetics, Baylor College of Medicine, One Baylor Plaza, Houston, Texas 770301, USA.
Correspondence to: Ype Elgersma1 e-mail: y.elgersma@erasmusmc.nl
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