LUZP deficiency affects neural tube closure during brain development

Biochem Biophys Res Commun. 2008 Nov 21;376(3):466-71. doi: 10.1016/j.bbrc.2008.08.170. Epub 2008 Sep 16.


LUZP is a leucine zipper-containing protein predominantly expressed in the brain. The functional significance of LUZP remains unknown. To explore the role of LUZP in brain development, a knockout mouse strain with a lacZ knock-in (Luzp-KO/lacZ-KI) has been established. LacZ reporter expression driven by the endogenous Luzp promoter was detected in the neuroepithelium and the cardiac tissue. Luzp(-/-) mice exhibited perinatal death, presumably due to the accompanied complex cardiovascular defects. Luzp(-/-) embryos displayed a cranial neural tube closure defect (NTD), with exposed brain tissues. Ectopic expression of Sonic-hedgehog, which is a protein known to be involved in neural tube closure, and elevated apoptosis were observed in the dorsal lateral neuroepithelium of the NTD Luzp(-/-) hindbrain. These findings assign a novel function of LUZP in the embryonic development of brain.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Apoptosis
  • Brain / cytology
  • Brain / embryology*
  • Brain / metabolism
  • DNA-Binding Proteins / genetics
  • DNA-Binding Proteins / physiology*
  • Hedgehog Proteins / biosynthesis
  • Mice
  • Mice, Knockout
  • Neural Tube / abnormalities*
  • Neural Tube / metabolism
  • Oligonucleotide Array Sequence Analysis
  • Rhombencephalon / cytology
  • Rhombencephalon / embryology
  • Rhombencephalon / metabolism


  • DNA-Binding Proteins
  • Hedgehog Proteins
  • Luzp1 protein, mouse
  • Shh protein, mouse