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


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.

Pubmed ID: 15632069


  • Steele-Perkins G
  • Plachez C
  • Butz KG
  • Yang G
  • Bachurski CJ
  • Kinsman SL
  • Litwack ED
  • Richards LJ
  • Gronostajski RM


Molecular and cellular biology

Publication Data

January 5, 2005

Associated Grants

  • Agency: NIDDK NIH HHS, Id: DK48796
  • Agency: NIDDK NIH HHS, Id: DK58401
  • Agency: NICHD NIH HHS, Id: HD34901
  • Agency: NHLBI NIH HHS, Id: HL60907
  • Agency: NICHD NIH HHS, Id: R01 HD034908

Mesh Terms

  • Agenesis of Corpus Callosum
  • Animals
  • Biological Markers
  • Brain
  • Cell Differentiation
  • Embryo, Mammalian
  • Female
  • Gene Targeting
  • Gestational Age
  • Humans
  • Lung
  • Mice
  • Mice, Inbred C57BL
  • NFI Transcription Factors
  • Pregnancy
  • Proteins
  • Recombinant Fusion Proteins