The transcription factor gene Nfib is essential for both lung maturation and brain development

George Steele-Perkins, Céline Plachez, Kenneth G. Butz, Guanhu Yang, Cindy J. Bachurski, Stephen L. Kinsman, E. David Litwack, Linda J. Richards, Richard M. Gronostajski

Research output: Contribution to journalArticlepeer-review

246 Scopus citations


The phylogenetically conserved nuclear factor I (NFI) gene family encodes site-specific transcription factors essential for the development of a number of organ systems. We showed previously that Nfia-deficient mice exhibit agenesis of the corpus callosum and other forebrain defects, whereas Nfic-deficient mice have agenesis of molar tooth roots and severe incisor defects. Here we show that Nfib-deficient mice possess unique defects in lung maturation and exhibit callosal agenesis and forebrain defects that are similar to, but more severe than, those seen in Nfia-deficient animals. In addition, loss of Nfib results in defects in basilar pons formation and hippocampus development that are not seen in Nfia-deficient mice. Heterozygous Nfib-deficient animals also exhibit callosal agenesis and delayed lung maturation, indicating haploinsufficiency at the Nfib locus. The similarity in brain defects in Nfia- and Nfib-deficient animals suggests that these two genes may cooperate in late fetal forebrain development, while Nfib is essential for late fetal lung maturation and development of the pons.

Original languageEnglish
Pages (from-to)685-698
Number of pages14
JournalMolecular and cellular biology
Issue number2
StatePublished - Jan 2005


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