Lissencephaly: mechanistic insights from animal models and potential therapeutic strategies.

@article{WynshawBoris2010LissencephalyMI,
  title={Lissencephaly: mechanistic insights from animal models and potential therapeutic strategies.},
  author={Anthony Wynshaw-Boris and Tiziano Pramparo and Yong Ha Youn and Shinji Hirotsune},
  journal={Seminars in cell & developmental biology},
  year={2010},
  volume={21 8},
  pages={823-30}
}
Lissencephaly is a severe human neuronal migration defect characterized by a smooth cerebral surface, mental retardation and seizures. The two most common genes mutated in patients with lissencephaly are LIS1 and DCX. LIS1 was the first gene cloned that was important for neuronal migration in any organism, and heterozygous mutations or deletions of LIS1 are found in the majority of patients with lissencephaly, while DCX mutations were found in males with X-linked lissencephaly. In this review… CONTINUE READING