Monkeying around: Monkeys carrying mutations in MeCP2 show some features of Rett syndrome that are not found in mice that model the syndrome.
Monkeys with a mutation in the Rett syndrome gene, MeCP2, show characteristics of the condition, such as sleep problems and sensory sensitivity, as well as social difficulties and repetitive behaviors reminiscent of autism1.
The monkeys also show features that are difficult to assess in mouse models of Rett, such as trouble recognizing another monkey’s facial expressions.
The findings suggest that the monkeys may help researchers understand the neural underpinnings of the syndrome, and test treatments for the condition.
“This data can’t be gotten from rodents,” says lead investigator Yongchang Chen, professor of developmental biology at Kunming University of Science and Technology in China.
Rett syndrome mainly affects girls because most boys with a MeCP2 mutation are stillborn or die shortly after birth. The girls develop typically for about 18 months and then regress, losing their language and motor skills. Roughly 60 percent have autism2.
Male monkeys with a MeCP2 mutation are also stillborn, the study shows. Females show signs of Rett syndrome and autism, but it is not yet known whether they regress. In eye-tracking tests, they seem to have trouble recognizing the emotions of other monkeys — a key feature of primate social behavior that is absent in rodents. The results were published 18 May in Cell.
“Primate models are going to be very important for understanding how these disorders unfold,” says Michael Platt, professor of neuroscience at the University of Pennsylvania in Philadelphia, who was not involved in the study. “You can measure how much time a mouse spends sleeping or next to another mouse, but here they have the opportunity to really look at one of the major behavioral impairments [of autism].”
Chen and his colleagues described the first of their MeCP2…
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