Similar to the well-known critical period in development for language acquisition, there is likely to be a critical window during childhood for acquiring normal social behavior. An important goal in autism research is to identify this critical period, and possibly to manipulate the length of the window or reopen it in adulthood to help with the acquisition and maintenance of appropriate social behaviors.
Ype Elgersma and his colleagues at Erasmus University Medical Center in the Netherlands are planning to address this topic using a mouse model of Angelman syndrome, a severe neurodevelopmental disorder. Many people with Angelman syndrome fulfill some (or all) of the criteria for autism. Dysfunction of the maternally inherited gene UBE3A is responsible for the syndrome, yet the precise role of UBE3A in brain development and function is poorly understood.
Elgersma’s lab has developed a unique inducible mouse model for Angelman syndrome. The mice are born with the UBE3A gene silenced, but the researchers can turn the gene back on at any time. They plan to use these mice to determine the critical window during development for establishing autism-related Angelman syndrome behaviors, such as social interaction deficits and repetitive or inflexible behavior.
These behavioral findings could then be compared with changes in neuronal function of the prefrontal cortex, a brain area thought to play a key role in social behavior. The results may shed light on the role of UBE3A in brain development and provide insight into the mechanisms and critical periods underlying normal social behavior.
