Rescue of neurological deficits in a mouse model for Angelman syndrome by reduction of αCaMKII inhibitory phosphorylation
GM Van Woerden, KD Harris, MR Hojjati… - Nature …, 2007 - nature.com
GM Van Woerden, KD Harris, MR Hojjati, RM Gustin, S Qiu, R de Avila Freire, Y Jiang…
Nature neuroscience, 2007•nature.comAngelman 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.
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.
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.
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