Familial dysautonomia model reveals Ikbkap deletion causes apoptosis of Pax3+ progenitors and peripheral neurons.

@article{George2013FamilialDM,
  title={Familial dysautonomia model reveals Ikbkap deletion causes apoptosis of Pax3+ progenitors and peripheral neurons.},
  author={Lynn George and Marta E. Chaverra and Lindsey Wolfe and Julian Thorne and Mattheson Close-Davis and Amy Eibs and Vickie Riojas and Andrea Grindeland and Miranda E Orr and George A. Carlson and Frances Lefcort},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  year={2013},
  volume={110 46},
  pages={18698-703}
}
Familial dysautonomia (FD) is a devastating developmental and progressive peripheral neuropathy caused by a mutation in the gene inhibitor of kappa B kinase complex-associated protein (IKBKAP). To identify the cellular and molecular mechanisms that cause FD, we generated mice in which Ikbkap expression is ablated in the peripheral nervous system and identify the steps in peripheral nervous system development that are Ikbkap-dependent. We show that Ikbkap is not required for trunk neural crest… CONTINUE READING
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