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


Journal Article

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 alphaCaMKII. 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 alphaCaMKII.

Full Text

Cited Authors

  • van Woerden, GM; Harris, KD; Hojjati, MR; Gustin, RM; Qiu, S; de Avila Freire, R; Jiang, Y-H; Elgersma, Y; Weeber, EJ

Published Date

  • March 2007

Published In

Volume / Issue

  • 10 / 3

Start / End Page

  • 280 - 282

PubMed ID

  • 17259980

Pubmed Central ID

  • 17259980

Electronic International Standard Serial Number (EISSN)

  • 1546-1726

International Standard Serial Number (ISSN)

  • 1097-6256

Digital Object Identifier (DOI)

  • 10.1038/nn1845


  • eng