Israeli scientist discovers William Syndrome is effected by Myelination

Conducted by a Tel Aviv University researcher, the study leads to hope that future drug therapy might ease some of the symptoms of the syndrome.

By
April 25, 2019 04:56
1 minute read.
mice

Mice [Illustrative]. (photo credit: INGIMAGE)

 
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Dr. Boaz Barak of Tel Aviv University led a recent study that discovered the relationships between William Syndrome and myelin sheaths.

Mylein covers axons, which carry electrical impulses in neurons that enable them to communicate. The study suggests that some of the difficulties experienced by those with William Syndrome are the result of deficient myelin.

William Syndrome is a rare genetic disease that makes those who suffer from it increasingly social despite having mild mental disabilities. They often smile a lot and display rich vocabulary, which might confuse people who aren’t aware of their real abilities.

To examine the hypothesis, Barak removed gene Gtf2i from excitatory neurons in the mouse fore-brains hoping to create William Syndrome in his long-tailed test subjects. The mice showed more interest in strange mice than the control population of mice and had difficulty with skills tests.

“Seventy percent of the genes significantly down regulated by the neuronal Gtf2i deletion were related to myelin," he said in a press release.

As myelin is also related to other diseases such as multiple sclerosis, it was possible that drugs that treat it might benefit those suffering from William Syndrome.


Two drugs were tried: 4-AP and Clemastine. The first blocks axonal electrical signal leakages, while the other normalizes the thickness of the myelin sheaths around the axons.

The mutant mice improved their social behavior, meaning they found as much interest in strange mice as healthy mice do once they began to experience normal myelin levels, Barak explained. 

"If we understand how to control myelination abnormalities, perhaps our understanding can help WS, MS and even certain autism patients," he said.

The study was largely conducted in Prof. Guoping Feng's laboratory at MIT.

 
  

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