Basement membrane fragility underlies embryonic lethality in fukutin-null mice.

@article{Kurahashi2005BasementMF,
  title={Basement membrane fragility underlies embryonic lethality in fukutin-null mice.},
  author={Hiroki Kurahashi and Mariko Taniguchi and Chikara Meno and Yoshihiro Taniguchi and Satoshi Takeda and Masato Horie and Hiroki Otani and Tatsushi Toda},
  journal={Neurobiology of disease},
  year={2005},
  volume={19 1-2},
  pages={208-17}
}
Fukuyama-type congenital muscular dystrophy (FCMD), associated with brain malformation due to defects in neuronal migration, is caused by mutations in fukutin. Several lines of evidence suggest that the fukutin protein plays a pivotal role in synthesis of O-mannosyl sugar moieties of alpha-dystroglycan, a cell surface laminin receptor. Here, through targeted disruption of the orthologous mouse fukutin gene, we show that the fukutin protein is essential, as homozygous-null embryos die by E9.5 of… CONTINUE READING

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