Reversing Rett Syndrome in mice

5 July 2011

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Category: Research & medical benefits

mice–mouse–test–tube.jpgNew research using mouse models has shown that glial cells, which protect and support neurons in the brain, play a central role in preventing the severe symptoms of Rett Syndrome.

Rett Syndrome is a debilitating autism spectrum disorder that occurs almost exclusively in girls and symptoms are visible by 18 months. Loss of speech, motor control and repetitive hand movements are characteristic of the disease. Seizures and severe digestive complications are also typical symptoms of the syndrome. Most patients of the disease live to adulthood and require full-time care.

Rett Syndrome is caused by mutations in the gene coding for the protein MeCP2. It was previously thought that the disease was exclusively caused by a loss of MeCP2 function in neurons but this most recent research has demonstrated that these mutations also occur in glial cells.

When these mutations to MeCP2 are induced in mice they demonstrate symptoms with characteristics similar to those expressed in humans with Rett Syndrome. Therefore mice are effective models for studying the disease. By inducing re-expression of MeCP2 solely in the mouse astrocytes, a particular type of glial cell, researchers were able to reverse Rett symptoms.

Further research will look into whether other types of glial cell play a role in Rett Syndrome and investigate how astrocytes interact with neurons. It is hoped that the knowledge gained from further investigation will open up new treatment possibilities for Rett suffers.