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ScienceDaily (June 2, 2011) — The protein MeCP2 is porridge to the finicky neuron. Like Goldilocks, the neuron or brain cell needs the protein in just the right amount. Girls born with dysfunctional MeCP2 (methyl-CpG-binding protein 2) develop Rett syndrome, a neurological disorder. Too much MeCP2 can cause spasticity or developmental delay with autism-like symptoms in boys.


Now, researchers at Baylor College of Medicine and Texas Children’s Hospital have found that the neuron needs a steady supply of this protein for its entire existence. A report on this research appears online in Science Express.


MeCP2 was found in 1999 in the laboratory of Dr. Huda Zoghbi, director of the Jan and Dan Duncan Neurological Research Institute at TCH and professor of neurology, neuroscience, pediatrics and molecular and human genetics at BCM and a Howard Hughes Medical Institute Investigator. A mutation in MeCP2 results in Rett syndrome, a neurological disorder that strikes mainly girls. Male fetuses born with the mutation (which results in dysfunctional protein) die before birth, but girls appear normal until they are between 6 and 18 months. Then they begin to regress and their growth slows. They develop abnormal hand motions such as wringing. Their crawling and walking regresses and they eventually lose the ability to speak or communicate. They exhibit some symptoms of autism.


Clearly, MeCP2 is critical to normal mental functioning, but a question remained. Do neurons need MeCP2 throughout life or would they be protected and work properly if MeCP2 is provided only early in life and then discontinued during adulthood?



Journal Reference:


  1. Christopher M. McGraw, Rodney C. Samaco, and Huda Y. Zoghbi. Adult Neural Function Requires MeCP2. Science, 2011; DOI: 10.1126/science.1206593

 

Read in Full:

http://www.sciencedaily.com/releases/2011/06/110602143204.htm



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