TRANSLATION DYSREGULATION IN AUTISM SPECTRUM DISORDERS

Mutations in several negative regulators of translation initiation cause developmental disabilities and autism spectrum disorders in humans, including fragile X syndrome and tuberous sclerosis complex. Mouse models have been developed for several of these disorders, and they exhibit altered translation control, abnormal synaptic function, and aberrant behavior. Findings from recent studies of mice that model fragile X syndrome, Wolcott-Rallison syndrome, and non-syndromic autism will be discussed in this presentation. These studies have revealed interesting links among the biochemical activities of translation factors, synaptic function, and behavior, and provide insight into the molecular basis of developmental disabilities and autism spectrum disorders.