Ectopia of meningeal fibroblasts and reactive gliosis in the cerebral cortex of the mouse model of muscle-eye-brain disease.

@article{Yang2007EctopiaOM,
  title={Ectopia of meningeal fibroblasts and reactive gliosis in the cerebral cortex of the mouse model of muscle-eye-brain disease.},
  author={Yuan Yuan Yang and Peng Zhang and Yufang Xiong and Xiaofeng Li and Yue Qi and Huaiyu Hu},
  journal={The Journal of comparative neurology},
  year={2007},
  volume={505 5},
  pages={459-77}
}
Congenital muscular dystrophies with brain malformations, such as muscle-eye-brain disease, exhibit neural ectopias caused by overmigration of neurons. Such overmigration is evident in protein O-mannose beta-1,2-N-acetylglucosaminyltransferase (POMGnT1) knockout mouse, a model of muscle-eye-brain disease, caused by breaches in the pial basement membrane. We hypothesize that breaches in pial basement membrane disrupt the neural-meningeal boundary, resulting in ectopia of meningeal fibroblasts in… CONTINUE READING

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