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

LUZP deficiency affects neural tube closure during brain development.

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Institute of Neuroscience, School of Life Science, National Yang-Ming University, 155, Section 2, Linong Street, Taipei 11211, Taiwan.


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.

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